submucous cleft palate

粘膜下腭裂
  • 文章类型: Journal Article
    背景:粘膜下腭裂(SMCP)是一种先天性异常,其特征是存在Calnan三联征。然而,在临床实践中,对于个人来说,在三合会中表现出一个或两个解剖异常是很常见的。此外,SMCP的定义在文献中一直是多样和模糊的。因此,本研究旨在分析解剖学异常与咽喉功能不全(VPI)发生的相关性。
    方法:我们对2012年1月至2023年6月因常规口腔检查中发现的语音问题或解剖学异常而转诊到我们诊所的99例患者进行了回顾性分析。一名外科医生进行了所有体检。我们评估了骨性缺口的存在,透明带,和双歧小舌,给每个异常分配一个分数,并分析了它们与VPI的相关性。检查了每种异常与VPI发展的相关性,随着异常数量与VPI的关系。
    结果:在99名患者中,27例确诊为VPI。只有骨性缺口与VPI发育有显著相关性。随着更多解剖异常的存在,VPI的发生率趋于增加。VPI发生在所有三种解剖异常的患者中约40%。
    结论:研究结果强调了对SMCP患者进行细致的口内检查以及对有骨性切迹或两个或两个以上解剖异常的患者进行仔细监测的重要性。
    BACKGROUND: Submucous cleft palate (SMCP) is a congenital anomaly characterized by the presence of Calnan\'s triad. However, in clinical practice, it is common for individuals to exhibit one or two anatomical abnormalities within the triad. Furthermore, the definition of SMCP has been diverse and ambiguous in literature. Therefore, this study aimed to analyze the correlation between anatomical abnormalities and development of velopharyngeal insufficiency (VPI).
    METHODS: We conducted a retrospective analysis of 99 patients referred to our clinic for speech issues or anatomical abnormalities identified during routine oral examinations from January 2012 to June 2023. A single surgeon performed all physical examinations. We evaluated the presence of bony notch, zona pellucida, and bifid uvula, assigned a score to each abnormality, and analyzed their correlation with VPI. The correlation of each of the abnormalities with VPI development was examined, along with the relationship between the number of abnormalities and VPI.
    RESULTS: Among the 99 patients, 27 were diagnosed with VPI. Only the bony notch had a significant correlation with VPI development. The incidence of VPI tended to increase with the presence of more anatomical abnormalities. VPI occurred in approximately 40% of patients exhibiting all three anatomical abnormalities.
    CONCLUSIONS: The study findings highlight the importance of meticulous intraoral examinations in patients with SMCP and careful monitoring of patients with a bony notch or two or more anatomical abnormalities.
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  • 文章类型: Journal Article
    目的:这项研究的目的是通过头颅测量评估未手术的成人粘膜下腭裂(SMCP)的咽部形态,成人未手术的明显腭裂(OCP),和没有裂缝的成年人。
    方法:本研究采用回顾性横断面设计。对三组成年人进行了侧位头颅X线摄影:1)29例未经修复的SMCP;2)41例未经修复的OCP;3)39例无裂,作为控制。单因素方差分析和秩和检验用于组间比较。P值设定在0.05。
    结果:未手术的SMCP和OCP受试者的软腭长度和软腭长度与咽部深度之比明显低于非裂对照组。在咽部深度也观察到显著差异,鼻咽深度,未经手术的OCP和非left裂对照的受试者之间的咽后壁厚度。
    结论:有裂隙和没有裂隙的个体之间的咽部形态有显著差异,特别是软腭长度和软腭长度与咽部深度之比。
    OBJECTIVE: The aim of this study was to cephalometrically evaluate the pharyngeal morphology in adults with unoperated Submucous Cleft Palate (SMCP), adults with unoperated Overt Cleft Palate (OCP), and adults without clefts.
    METHODS: This study employed a retrospective cross-sectional design. Lateral cephalometric radiography was performed on three groups of adults: 1) 29 with unrepaired SMCP; 2) 41 with unrepaired OCP; and 3) 39 without clefts, who served as controls. One-way ANOVA and rank-sum tests were used for intergroup comparisons. P value was set at .05.
    RESULTS: The soft palate length and the ratio of soft palate length to pharyngeal depth were significantly lower in subjects with unoperated SMCP and OCP than in non-cleft controls. Significant differences were also observed in pharyngeal depth, nasopharyngeal depth, and posterior pharyngeal wall thickness between subjects with unoperated OCP and non-cleft controls.
    CONCLUSIONS: Pharyngeal morphology differs significantly between individuals with and without clefts, particularly in soft palate length and the ratio of soft palate length to pharyngeal depth.
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  • 文章类型: Journal Article
    Objective: To determine which surgical technique offers the lowest rate of velopharyngeal insufficiency (VPI) without the need for further operative intervention, in pediatric patients with nonsyndromic submucous cleft palate (SMCP). Methods: This systematic review and meta-analysis included articles reporting on nonsyndromic pediatric patients treated surgically during childhood for SMCP, with data on postoperative speech outcomes and/or recommendations for secondary surgery. Main outcome measures included rates of unfavorable speech outcomes defined as persistent VPI requiring secondary surgery and speech outcome data. Results: 15 articles met our inclusion criteria, reporting on 383 children who underwent surgical treatment; 343 patients were included in studies reporting recommendations for secondary surgery. There was 1 randomized comparative trial, 4 comparative studies, and 10 single cohort studies. Eight articles used validated speech assessment tools. Our model showed the proportion of patients recommended for secondary surgery varied between techniques, ranging from 0.0% (CI 0.0, 1000) in pharyngeal flap to 17.8% (CI 8.9, 32.5) in straight line repair techniques, but there was no statistically significant difference between treatments (P = .33). Speech improvement ranged from 44.4% to 100%, with 9 studies recommending secondary surgery for some of their patient series. Conclusions: Although not of statistical significance, pharyngeal flap yields the lowest rate of reoperation as a primary technique for pediatric patients with nonsyndromic SMCP. Delayed repair age inherent to SMCP may render operations that rely on a functional levator muscle with less favorable outcomes. The absence of standardized surgical techniques, speech outcomes, speech therapy, and assessment make comparative analysis and recommendation difficult. We advocate for standardized speech assessment tools to improve future quantitative assessment of cleft surgery outcomes and a randomized controlled trial to better elucidate the preferred first-line technique.
    Objectif: Déterminer les techniques chirurgicales qui offrent le plus bas taux d’insuffisance vélopharyngée (IVP) sans autre intervention opératoire chez les patients pédiatriques présentant une fissure palatine sous-muqueuse (FPSM) non syndromique. Méthodologie: La présente analyse systématique et méta-analyse incluait des articles rendant compte de patients pédiatriques non syndromiques ayant reçu un traitement chirurgical pendant l’enfance à cause d’une FPSM, y compris des données sur l’élocution postopératoire ou les recommandations en vue d’une opération secondaire. Les principales mesures de résultats incluaient les taux d’élocution défavorables définis comme une IVP persistante exigeant une opération secondaire et les données sur les résultats de l’élocution. Résultats: Au total, 15 articles respectaient les critères d’inclusion et rendaient compte de 383 enfants qui ont subi un traitement chirurgical; 343 patients ont participé à des études qui recommandaient une opération secondaire. Ces articles incluaient une étude comparative randomisée, quatre études comparatives et dix études de cohortes uniques. Huit faisaient appel à des outils d’évaluation de l’élocution validés. Le modèle des auteurs démontrait que la proportion de patients chez qui on recommandait une opération secondaire variait selon les techniques, soit de 0,0 % (IC, 0,0, 100,0) pour la technique de lambeau pharyngien à 17,8 % (IC, 8,9, 32,5) pour la technique de réparation linéaire, mais il n’y avait pas de différence significative entre les traitements (p=0,33). L’amélioration de l’élocution oscillait entre 44,4 % et 100 %, neuf études recommandant une opération secondaire pour certains patients de leur série. Conclusions: Même si ce résultat n’avait pas de signification statistique, le lambeau pharyngé est associé au taux de réopération le plus faible lorsqu’il est utilisé comme technique primaire chez les patients pédiatriques ayant une FPSM non syndromique. En raison de l’âge tardif de réparation inhérent à la FPSM, les opérations qui reposent sur le muscle élévateur fonctionnel peuvent donner des résultats moins favorables. Il peut être difficile de procéder à une analyse comparative et de formuler des recommandations à cause de l’absence de techniques chirurgicales standardisées, de résultats sur l’élocution, d’orthophonie et d’évaluation. Les auteurs préconisent des outils d’évaluation de l’élocution standardisés pour améliorer la future évaluation quantitative des résultats de l’opération de la fissure palatine et la tenue d’une étude contrôlée randomisée pour mieux déterminer la technique de première ligne à favoriser.
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  • 文章类型: Journal Article
    目的:最近的研究表明,与普通人群相比,患有孤立性唇裂(CL)的儿童更容易发生中耳疾病和咽鼓管功能障碍(ETD)。这可能与异常的pal肌系统或未诊断的粘膜下c裂(SMCP)有关。我们的目的是确定出现ETD的CL患者中SMCP的患病率。
    方法:在学术三级医疗中心,对需要鼓膜造口术的孤立性CL患儿进行了20年的回顾性分析。人口统计,临床,并收集手术数据。
    结果:三百十二名患者患有孤立的CL,29名(9.3%)儿童需要鼓膜置管。其中,9人(31%)被发现患有SMCP(7名男性,6高加索人)。CL修复的平均年龄为3.94±1.03个月,鼓膜置管的平均年龄为13.68±13.8个月。所有9名患者都患有慢性中耳炎,其中四个患有轻度传导性听力损失,三个患有中度传导性听力损失。在CL诊断时诊断出SMCP(4),在CL诊断与慢性中耳炎/ETD(2)和慢性中耳炎/ETD诊断后。
    结论:孤立性唇裂患者的中耳疾病或咽鼓管功能障碍应引起对伴随的未确诊SMCP的怀疑。
    OBJECTIVE: Recent studies have suggested that children with an isolated cleft lip (CL) are more likely to develop middle ear disease and eustachian tube dysfunction (ETD) compared to the general population. This may be related to abnormal palatal musculature or an undiagnosed submucosal cleft palate (SMCP). We aim to determine the prevalence of SMCP in patients with CL who exhibit ETD.
    METHODS: A retrospective chart review was performed for children with an isolated CL requiring tympanostomy tubes over a 20-year period at an academic tertiary care medical center. Demographic, clinical, and surgical data were collected.
    RESULTS: Three hundred twelve patients had an isolated CL, and 29 (9.3 %) children required tympanostomy tubes. Of those, nine (31 %) were found to have a SMCP (7 males, 6 Caucasian). The average age at CL repair was 3.94 ± 1.03 months, and the average age at tympanostomy tube placement was 13.68 ± 13.8 months. All nine patients had chronic otitis media, with four having mild conductive hearing loss and three having moderate conductive hearing loss. The SMCP was diagnosed at the time of CL diagnosis (4), after CL diagnosis with the diagnosis of chronic otitis media/ETD (2) and after a diagnosis of chronic otitis media/ETD.
    CONCLUSIONS: Middle ear disease or eustachian tube dysfunction in a patient with an isolated cleft lip should raise suspicion for an accompanying undiagnosed SMCP.
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  • 文章类型: Journal Article
    背景:对于有症状的粘膜下腭裂患者,一旦证实咽喉功能不全,建议进行手术治疗。这项研究描述了微创腔内静脉成形术的程序和临床结果。
    方法:2013年8月至2017年3月,7名患者(中位年龄,36个月;范围,16-60个月,5名女性和2名男性)患有粘膜下left裂的患者接受了腔内静脉成形术。既不应用鼻粘膜切口也不应用外侧松弛切口。至少进行两次随访:一次在术后三周,一次在术后两到三年之间(平均,31个月;范围,26-35个月)。当患者至少3岁时,由言语病理学家评估言语。
    结果:没有出现口鼻瘘或面部发育明显障碍。所有7例患者均未表现出或表现出轻度的鼻塞和空气排放,并且有能力或至少有临界的主管咽喉功能。
    结论:眼球内成形术可以作为另一种治疗粘膜下腭裂伴咽喉功能不全的方法,导致令人满意的改善咽喉功能。因为既没有使用侧向切口也没有使用鼻切口,可以将面部生长的负担和口鼻瘘的风险降至最低。
    BACKGROUND: Surgical treatment is recommended for patients with symptomatic submucous cleft palate once velopharyngeal insufficiency has been proven. This study describes the procedure and clinical outcomes of minimally invasive intravelar veloplasty.
    METHODS: From August 2013 to March 2017, seven patients (median age, 36 months; range, 16-60 months, 5 females and 2 males) with submucous cleft palate underwent intravelar veloplasty. Neither a nasal mucosal incision nor a lateral relaxing incision was applied. Follow-up was performed at least twice: once at three weeks postoperatively and again between two and three years postoperatively (average, 31 months; range, 26-35 months). Speech was assessed by speech-language pathologists when the patients were at least 3 years old.
    RESULTS: There were no cases of oronasal fistula or noticeable disturbance of facial development. All seven patients showed no or mild hypernasality and air emission and competent or at least borderline competent velopharyngeal function.
    CONCLUSIONS: Intravelar veloplasty could serve as another option for managing submucous cleft palate with velopharyngeal insufficiency, resulting in satisfactory improvement in velopharyngeal function. Because neither a lateral nor a nasal incision was used, the burden of facial growth and risk of oronasal fistula can be minimized.
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  • 文章类型: English Abstract
    With a frequency of 1 per 500 live births, a cleft lip and palate is one of the most frequent congenital malformations. Untreated, it leads to disturbances in feeding, speech, hearing, tooth position and esthetics. A multifactorial genesis is assumed. The fusion of the different facial processes takes place in the first 3 months of pregnancy and a cleft can develop during this time. Surgical treatment includes the early anatomical and functional restoration of the affected structures within the first year of life in order to enable normal intake of food, articulation, nasal breathing and middle ear ventilation. Breastfeeding is possible in children with a cleft formation but alternative feeding methods, such as finger feeding, often have to be used. In addition to the surgery for primary closure of the cleft, otorhinolaryngological (ENT) interventions, speech therapy, orthodontic treatment as well as other surgical interventions are part of the interdisciplinary treatment concept.
    UNASSIGNED: Lippen-Kiefer-Gaumen-Spalten gehören mit 1:500 Lebendgeborenen zu den häufigsten angeborenen Fehlbildungen. Unbehandelt führen sie zu Störungen in Ernährung, Sprechen, Hören, Zahnstellung und Ästhetik. Von einer multifaktoriellen Genese wird ausgegangen. Die Verschmelzung der Gesichtsfortsätze findet in den ersten 3 Schwangerschaftsmonaten statt; in dieser Zeit kann eine Spalte entstehen. Die chirurgische Behandlung beinhaltet die anatomische und funktionelle Wiederherstellung der betroffenen Strukturen innerhalb des 1. Lebensjahres, um Nahrungsaufnahme, Lautbildung, Nasenatmung und Mittelohrbelüftung zu normalisieren. Stillen ist bei Kindern mit einer Spaltbildung möglich, oft muss aber auf alternative Fütterungsmethoden wie z. B. das „Finger feeding“ zurückgegriffen werden. Neben den Operationen zum primären Spaltverschluss sind HNO-Eingriffe, logopädische und kieferorthopädische Therapie sowie weitere chirurgische Interventionen Teil des interdisziplinären Behandlungskonzepts.
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  • 文章类型: Journal Article
    目的:评价解剖肌解剖修复对22q11.2缺失综合征粘膜下腭裂(SMCP)患者咽喉功能不全(VPI)的疗效。
    方法:对一名外科医生超过10年的管理进行回顾性盲法随机分析。
    方法:本研究在英国一家儿科专科医院进行。
    方法:SMCP和22q11.2缺失综合征的儿童。
    方法:所有参与者都接受了根治性肌肉剥离术。
    方法:术前和术后测量包括解剖缺损的严重程度,数字化的语音样本和横向图像,随机化,然后使用经过验证的技术进行外部和盲目分析。采用Stata软件进行统计分析。
    结果:57例22q11.2缺失综合征患儿被纳入本分析。术中,大多数病例被确定为SMCPI级异常.术后,鼻塞的显著改善,静息腭长度,最大闭合时的腭长度,观察到最大闭合时的腭偏移和间隙大小。到10年,59%的患者进行了二次手术。
    结论:肌肉剥离修复可改善鼻塞,22q11.2缺失综合征患者的腭闭合功能和闭合间隙。尽管超过50%的患者可能需要进一步手术,肌肉解剖修复应该是第一步,因为它在年轻的时候就很实用,当侵入性调查是不可能的时候,其发病率较低,语音和语言的好处或改变计划,以减少阻塞的二次手术,当它导致减少咽喉间隙和改善上颚活动时,即使没有实现足够的咽喉闭合。
    OBJECTIVE: To evaluate the benefit of anatomical muscle dissection repair for velopharyngeal insufficiency (VPI) in patients with submucous cleft palate (SMCP) with 22q11.2 deletion syndrome.
    METHODS: Retrospective blinded randomised analysis of a surgeon\'s management over 10 years.
    METHODS: The study was performed at a specialised Paediatric hospital in the United Kingdom.
    METHODS: Children with SMCP and 22q11.2 deletion syndrome.
    METHODS: All participants underwent radical muscle dissection veloplasty.
    METHODS: Pre- and post- operative measurements included severity of anatomical defect, speech samples and lateral images which were digitised, randomised then externally and blindly analysed using validated techniques. Stata software was used to perform statistical analysis.
    RESULTS: 57 children with 22q11.2 deletion syndrome were included in this analysis. Intra-operatively, the majority of cases were identified as SMCP Grade I anomalies. Post-operatively, a statistically significant improvement in hypernasality, resting palate length, palate length at maximum closure, palate excursion and gap size at maximum closure was observed. Secondary surgery was performed for 59% of patients by ten years.
    CONCLUSIONS: Muscle dissection repair improves hypernasality, palate closure function and the closure gap in patients with 22q11.2 deletion syndrome. Although over 50% of patients may require further surgery, muscle dissection repair should be a first step due to its utility at a younger age, when invasive investigations are impossible, its lower morbidity, speech and language benefits or altering the plans for less obstructive secondary surgery when it lead to reduced velo-pharyngeal gap and improved palate mobility even when adequate velo-pharyngeal closure was not achieved.
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  • 文章类型: Case Reports
    腭瘘是一个公认的并发症的个人谁已经接受了手术修复的腭裂,然而,先天性或特发性腭瘘很少见。本报告讨论了一名16岁女性粘膜下腭裂的表现和治疗,他最近出现了言语变化和新的腭瘘的证据。没有近期感染或已知创伤的病史,患者以前没有接受过任何腭手术。本报告讨论了临床表现,对这一罕见现象的管理和相关文献进行了推荐。
    Palatal fistulae are a recognised complication in individuals who have undergone surgical repair of a cleft palate, however, congenital or idiopathic palatal fistulae are rare. This report discusses the presentation and treatment of a 16-year-old female with a submucous cleft palate, who presented with a recent onset change in speech and evidence of a new palatal fistula. There was no history of recent infection or known trauma, and the patient had not undergone any previous palatal surgery. This report discusses the clinical presentation, recommended management and relevant literature for this rare phenomenon.
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  • 文章类型: Journal Article
    Ultrasound Tongue Imaging is increasingly used during assessment and treatment of speech sound disorders. Recent literature has shown that ultrasound is also useful for the quantitative analysis of a wide range of speech errors. So far, the compensatory articulations of speakers with cleft palate have only been analysed qualitatively. This study provides a pilot quantitative ultrasound analysis, drawing on longitudinal intervention data from a child with submucous cleft palate. Two key ultrasound metrics were used: 1. articulatory t-tests were used to compare tongue-shapes for perceptually collapsed phonemes on a radial measurement grid and 2. the Mean Radial Difference was reported to quantify the extent to which the two tongue shapes differ, overall. This articulatory analysis supplemented impressionistic phonetic transcriptions and identified covert contrasts. Articulatory errors identified in this study using ultrasound were in line with errors identified in the speech of children with cleft palate in previous literature. While compensatory error patterns commonly found in speakers with cleft palate have been argued to facilitate functional phonological development, the nature of our findings suggest that the compensatory articulations uncovered are articulatory in nature.
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  • 文章类型: Case Reports
    粘膜下腭裂(SMCP)患者遇到鼻咽息肉是一个难题,因为病变可以支持弱腭。去除该病变可能会掩盖SMCP,从而导致语音鼻音恶化。在文献中尚未报道过SMCP患者的鼻中隔息肉突出到鼻咽部。本报告描述了一个16岁的粘膜下left裂女孩的鼻中隔息肉,该息肉由鼻中隔的后边界引起,并在鼻咽中突出。息肉似乎支持弱腭,他们在发音时充当球窝。切除息肉可能会导致咽喉功能不全。在同一坐位中进行经鼻内窥镜内窥镜下切除息肉,并通过上方的咽瓣阻塞咽喉端口。使用听觉知觉评估和鼻测量的术前和术后言语评估显示鼻性没有恶化,患者还报告她的鼻呼吸有所改善。我们的结论是,SMCP患者鼻咽息肉的存在可以弥补言语问题。一次性咽瓣切除息肉和SMCP治疗是一种有效的方法,对患者的言语无不良影响。
    Encountering a nasopharyngeal polyp in a patient with submucous cleft palate (SMCP) is a difficult problem, as the lesion could support the weak palate. Removal of this lesion may unmask the SMCP with consequent worsening of speech nasality. Nasal septal polyp protruding to the nasopharynx in a patient with SMCP has not been reported before in the literature. This report describes a septal polyp arising from the posterior border of the nasal septum and protruding in the nasopharynx in a 16-year-old girl with submucous cleft palate. The polyp appeared to support the weak palate, and they acted as a ball and socket during speech articulation. Removal of this polyp may result in velopharyngeal insufficiency. Trans-nasal endoscopic removal of the polyp with obturation of the velopharyngeal port with a superiorly-based pharyngeal flap was performed in the same sitting. Pre- and postoperative speech evaluation using auditory perceptual assessment and nasometry revealed no worsening of nasality, also the patient reported improvement of her nasal breathing. We concluded that, the presence of a nasopharyngeal polyp in a patient with SMCP may compensate the speech problem. Removal of the polyp and treatment of SMCP by a pharyngeal flap in one-sitting is an effective procedure without adverse effect on patient\'s speech.
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