Validação do conteúdo de um protocolo de avaliação da motricidade orofacial em lactentes / Content validation of an orofacial myofunctional assessment protocol for infants / Validación del contenido de un protocolo para la evaluación de la motricidad orofacial en bebés

Introdução: Muitos estudos têm se dedicado a compreender melhor a dinâmica da avaliação das estruturas e funções estomatognáticas de lactentes; até a presente pesquisa não foram encontrados estudos específicos para essa faixa etária, até recentemente. Objetivo: Validar o conteúdo de um instrumento fonoaudiológico de avaliação da motricidade orofacial para lactentes na faixa etária de um mês a dois anos. Metodologia: Foi elaborado o instrumento para "avaliação fonoaudiológica da motricidade orofacial de lactentes de um mês a dois anos" a partir dos dados obtidos na literatura. A validação do conteúdo do instrumento se deu por meio da avaliação de quatro juízes para clareza dos itens propostos no protocolo e da representatividade dos mesmos no processo de validação do conteúdo. Os juízes classificaram cada item quanto à clareza, a partir de uma escala tipo Likert de quatro pontos, sendo: (4) muito claro, (3) claro, (2) pouco claro, (1) sem clareza, com o propósito de realizar a validação do conteúdo por meio da aplicação da equação do Índice de Validação do Conteúdo (IVC). Resultados: O protocolo desenvolvido possui 8 itens e uma breve anamnese: Hábitos Orais; Avaliação Estrutural; Respiração; Voz; Avaliação Funcional; Alimentação e Deglutição - líquidos e alimentos em pedaços; Diagnóstico Fonoaudiológico. A etapa seguinte contou com a análise da representatividade e para clareza dos itens do protocolo pelos juízes, e após a segunda análise, a validação do conteúdo resultou na permanência dos 8 itens com Índice de Validade de Conteúdo total de 100%. Conclusão: O conteúdo do protocolo foi considerado válido para uso na avaliação do público-alvo, comprovado por profissionais com experiência na área. A versão final do Protocolo de avaliação fonoaudiológica da motricidade orofacial de bebês foi finalizada com 8 itens de avaliação. (AU)

Introduction: The instruments for evaluating the structures and functions of the stomatognathic system in babies have been lacking in studies. Objective: To validate the content of a speech-language instrument to assess orofacial motricity for babies aged between one month and two years old. Methodology: The instrument for "speech-language assessment of the orofacial motricity of babies from one month to two years old" was created based on the data obtained by the integrative review. The instrument's content was validated through the evaluation of four judges. The judges classified each item according to clarity, based on a four-point Likert scale, as follows: (4) very clear, (3) clear, (2) lightly clear, (1) unclear, to perform content validation by applying the Content Validation Index (CVI) equation. Results: The developed protocol has eight items and a brief anamnesis: Oral Habits; Structural Assessment; Breathing; Voice; Functional Assessment; Feeding and Swallowing - liquids and food in pieces; and Speech-Language Diagnosis. The next step included the analysis of the representativeness of the protocol items by the judges. After the second analysis, the validation of the content resulted in the permanence of the eight items with a total Content Validity Index of 100%. Conclusion: The content of the protocol was considered valid for use in the evaluation of the target audience, proven by people with experience in the area. The final version of the Protocol for the Speech-Language Pathology Assessment of Orofacial Motricity in Babies was completed with eight assessment items. (AU)

Introducción: Los instrumentos para la evaluación de las estructuras y funciones del sistema estomatognático en los bebés han mostrado falta de estudios. Objetivo: Validar el contenido de un instrumento de fonoaudiología para la evaluación de la motricidad orofacial en bebés de un mes a dos años de edad. Metodología: Inicialmente, se llevó a cabo la elaboración del instrumento para la "evaluación logopédica de la motricidad orofacial de bebés de un mes a dos años de edad" propiamente dicho, a partir de los datos obtenidos por la revisión integradora. La validación del contenido del instrumento se realizó a través de la evaluación de cuatro jueces. Los jueces calificaron cada ítem en términos de claridad, utilizando una escala de Likert de cuatro puntos, de la siguiente manera: (4) muy claro, (3) claro, (2) poco claro, (1) poco claro, con el propósito de realizar la validación de contenido a través de la aplicación de la ecuación del Índice de Validación de Contenido (CVI). Resultados: después de la lectura y discusión de los artículos, fue posible desarrollar el protocolo que contiene 8 ítems y una breve anamnesis, que son: Hábitos Orales; Evaluación Estructural; Respiración; Voz; Evaluación Funcional; Alimentación y deglución: líquidos y alimentos en trozos; y; Diagnóstico de Patología del Habla-Lenguaje. El siguiente paso fue el análisis de la representatividad de los ítems del protocolo por parte de los jueces, y luego del segundo análisis, la validación de contenido resultó en la permanencia de 8 ítems con un Índice de Validez de Contenido total del 100%. Conclusión: El contenido del protocolo se consideró válido para su uso en la evaluación del público objetivo, confirmado por personas con experiencia en el área. La versión final del Protocolo de evaluación de la patología del habla y el lenguaje para la motricidad orofacial en bebés se completó con 8 ítems de evaluación. (AU)

Protocolos fonoaudiológicos de história clínica: revisão integrativa / Clinical history speech-language pathology protocols: integrative review

RESUMO Objetivo identificar, coletar e analisar, na literatura científica, evidências da existência de protocolos fonoaudiológicos de levantamento da história clínica, conforme classificação de risco, especialmente para distúrbios oromiofuncionais, em lactentes e pré-escolares. Estratégia de pesquisa foram selecionados estudos publicados, sem delimitação temporal, nas bases de dados eletrônicas LILACS, SciELO e PubMed e na literatura cinza (Google Acadêmico). Critérios de seleção estudos disponíveis na íntegra nas línguas portuguesa e inglesa, que identificassem protocolos fonoaudiológicos de história clínica aplicáveis a lactentes (6 a 23 meses de vida) e pré-escolares (24 a 71 meses de vida). Foram excluídas as revisões narrativas e de literatura (integrativa, sistêmica e escopo). Resultados foram encontradas 1371 publicações brasileiras no período de 1980 a 2022. Destas, foram identificadas apenas cinco que tratavam de protocolos fonoaudiológicos para levantamento de dados pregressos a partir da história clínica da faixa etária de 6 a 71 meses. Apenas um desses protocolos possuía classificação de risco para distúrbios fonoaudiológicos, distribuídos na área de linguagem e fluência. Os outros três eram da área de motricidade orofacial (MO) e não continham classificação de risco para distúrbio miofuncional orofacial. Conclusão existem poucos protocolos fonoaudiológicos para levantamento da história clínica de lactentes e pré-escolares que contenham, ou não, classificação de risco, publicados em revistas de acesso aberto e que passaram por processos completos de validação, sendo necessário ampliar estudos e publicações desses instrumentos, inclusive na área de MO.

ABSTRACT Purpose to identify, collect and analyze in the scientific literature evidence of the existence of speech therapy protocols for collecting clinical history, according to risk classification, especially for oromyofunctional disorders, in infants and preschoolers. Research Strategy We selected published studies, without temporal delimitation, in the electronic databases LILACS, SciELO and PUBMED; and in the gray literature (Google Academic). Selection criteria available in full in Portuguese and English, which identify speech-language pathology protocols of clinical history applicable to infants (6 to 23 months of age) and preschoolers (24 to 71 months of age). Narrative and literature reviews (integrative, systemic, and scope) were excluded. Results 1371 Brazilian publications were found in the period from 1980 to 2022. Of these, only five publications on speech therapy protocols were identified for collecting previous data from the clinical history of the age group between 6 and 71 months. Only two of these protocols have a risk classification for speech-language disorders, distributed in the areas of language and fluency. The other three are from the Orofacial Motricity (OM) area and do not carry a risk classification for orofacial myofunctional disorder. Conclusion There are few speech therapy protocols for surveying the clinical history of infants and preschoolers, whether or not they contain risk classification, published in open access journals that have a complete validation process.Therefore there is a need for more research and publication of these instruments, including in the area of OM.

Surgical Treatment of Oromandibular Limb Hypogenesis Syndrome Type I A by Distraction Osteogenesis Combined With Orthodontic Rehabilitation.

ABSTRACT: Oromandibular limb hypogenesis syndrome is a rare developmental anomaly and only a few cases are reported with complete surgical and orthodontic rehabilitation. An adult male patient with isolated hypoglossia, micrognathism, hypodontia, (oromandibular limb hypogenesis syndrome type I A) was treated with a combination of distraction osteogenesis and orthodontic intervention. The patient was followed up for the duration of 6 years from his first visit to 4 years after the surgery. The combined procedure resulted in successful and satisfactory treatment of the patient by restoring facial aesthetics, occlusal balance, and functional harmony. However, there was not enough tongue enlargement due to late surgical intervention. The objective of this report is to describe the etiology of hypoglossia, the consequences for oral function, and to share our experience from the oral rehabilitation during the treatment procedure.

Mapping of supernumerary elements in patients seeking orthodontic treatment

Dimension, number and shape alterations are frequently observed dental anomalies. Objective: The aim of this study was to map the frequency, the preferred location and the most prevalent types of supernumerary teeth, in the studied population. In addition, to assess the interference that supernumerary teeth may cause in the ectopic eruption of the normal dental series teeth. Methods: This is an observational retrospective study. Data collection was performed by evaluation of dental records of patients treated in an orthodontic clinic. Patients' radiographs, photographs and gypsum dental models were analyzed and data recorded in protocol files, by previously trained undergraduate and postgraduate students. Records from 1984 to 2019 were evaluated, and evaluation lasted two months. Data were stored in Microsoft Office Excel (version 2016) for descriptive analysis. Results: From the 920 patients' records analyzed, 2.7% presented at least one supernumerary tooth, with preferred location on the anterior maxillary area and mandibular parapremolar area. The most prevalent type was the supplemental tooth, with normal eruption orientation and normal alveolar location. Conclusion: The supernumerary teeth of the studied population showed little interference in the ectopic eruption of the normal dental series. Most of the supernumerary teeth were impacted, and, approximately one third of the supernumerary teeth showed deviated eruptions.

Introdução: Alterações de dimensão, número e forma são anomalias dentárias comumente observadas. Objetivo: O objetivo foi mapear a frequência de dentes supranumerários na população estudada, sua localização preferida e os tipos mais prevalentes. Além disso, avaliar a interferência que os dentes supranumerários podem causar na erupção ectópica da série dental normal. Métodos: A pesquisa possui desenho retrospectivo observacional. A coleta de dados foi realizada pela avaliação de prontuários odontológicos dos pacientes atendidos em uma clínica ortodôntica. Foram analisados radiografias, fotografias e modelos de gesso dental dos pacientes e as informações foram registradas em arquivos de protocolo do estudo. A coleta de dados foi realizada por uma estudante de graduação e uma da pós-graduação, previamente calibradas. A coleta durou dois meses e foram avaliados registros de 1984 a 2019. Os dados foram tratados no programa Microsoft Office Excel (version 2016) com análise descritiva dos dados. Resultados: Dos 920 prontuários analisados, 2,7% apresentavam pelo menos um dente supranumerário, com localização preferida na área superior anterior e na área parapremolar mandibular. O tipo mais prevalente foi o dente suplementar, com orientação de erupção normal e localização alveolar normal. Conclusão: Os dentes supranumerários da população estudada neste estudo mostraram pouca interferência na erupção ectópica da série dental normal. A maioria dos dentes supranumerários estava em posição de impactação. E, aproximadamente um terço dos dentes supranumerários apresentaram desvios de erupção.

Orthognathic Hardware Complications in the Era of Patient-Specific Implants.

BACKGROUND: Patients undergoing orthognathic skeletal correction present with a variety of comorbidities that may affect surgical outcomes. The purpose of this study was to determine how patient risk factors and operative technique contribute to complication rates after orthognathic surgery in the era of patient-specific implants. METHODS: Retrospective cohort analysis was conducted of pediatric patients undergoing Le Fort I osteotomy, bilateral sagittal split osteotomy, and/or genioplasty from 2014 to 2018. Patient risk factors, operative characteristics, and postoperative outcomes were gathered and compared with appropriate statistics. RESULTS: Ninety-four patients met inclusion criteria, with an overall 1-year complication rate of 11.7 percent (11 of 94). Patient-specific mandibular plates are significantly associated with infection (p = 0.009; OR, 8.8), occurrence of any complication (p = 0.003; OR, 8.3), readmission (p < 0.001; OR, 11.1), and reoperation (p < 0.001; OR, 11.4). In patients with syndromes or history of cleft lip/palate, patient-specific mandibular plates are associated with infection (p = 0.006; OR, 10.3), readmission (p < 0.001; OR, 21.6), and reoperation (p < 0.001; OR, 22.9). In multivariate regression controlling for age, sex, syndrome status, and orofacial cleft history, use of patient-specific mandibular plates was associated with infection (p = 0.017; adjusted OR, 12.5), any complication (p = 0.007; adjusted OR, 11.8), readmission (p = 0.001; adjusted OR, 17.9), and reoperation (p = 0.001; adjusted OR, 18.9). CONCLUSIONS: In the era of patient-specific orthognathic surgery, syndromic status and use of patient-specific mandibular plates are associated with increased infection, readmission, and reoperation because of hardware-related complications. The authors' data support increased caution and counseling with use of patient-specific mandibular implants in patients with syndromic status, history of orofacial cleft, and history of previous maxillomandibular surgery given increased risk of hardware-related complications. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Radiographic review of anatomy and pathology of the masticator space: what the emergency radiologist needs to know.

The differential diagnosis of a masticator space (MS) lesion is broad, owing in part to the multiple structures contained within such a small region. It is also because the MS is adjacent to many of the other deep spaces within the head and neck, which can act as gateways for disease spread. Therefore, emergency radiologists must be familiar with anatomy of the MS, as well as adjacent spaces in order to provide an accurate diagnosis to the referring clinician. This article illustrates the anatomy and common pathologies within the MS using a case-based multimodality approach. Common masticator space pathologies can be categorized into inflammatory/infectious, neoplastic, and vasoformative lesions. Important imaging features of MS lesions and patterns of disease spread will be discussed, with the aim of making this complex deep space more approachable in the emergent setting.

Evaluation of Canine Transpositions and Related Dental Anomalies in a Turkish Pediatric Population

Abstract Objective: To investigate the prevalence and characteristics of canine transpositions and to evaluate associated dental anomalies in a pediatric population. Material and Methods: This study was carried out by evaluating the panoramic radiographs of 11.750 patients ranging in ages from 6 to 17 presented to a pedodontic clinic over 5 years. The patients with canine transposition were included in the study. Age at diagnosis, gender, classification and location of transposition, presence of other dental anomalies were recorded. Differences were analyzed using the Chi-squared test. Results: Canine transposition was detected in 73 teeth from 69 patients (0.6%). Transpositions were more common in girls than in boys (55.1%). Transposed canine teeth were more frequently observed unilaterally (89%). The most commonly found transposition type was type 2 (42%). A total of 49.3% of 69 patients had retained primary canine teeth, 20.3% had congenitally missing teeth, and 11.6% had peg-shaped, microdont lateral incisors. Impacted teeth excluding third molars and taurodontism were also observed. Conclusion: Although the canine transpositions are generally asymptomatic in children in the period of growth and development, the importance of early diagnosis is crucial to prevent various clinical problems.

Intervalo de confianza para las normas del cefalograma resumido de Ricketts en niños de cubanos / Confidence interval for the standards of the Ricketts´summarized cephalogram in Cuban children

Introducción: en hallazgos cefalométricos de diferentes países en poblaciones con patrones de crecimientos y oclusión normal, se reportan diferencias estadísticamente significativas a los valores dados en los cefalogramas de Ricketts, Steiner, Downs, Jarabak y otros. En Cuba no existen estudios de estos patrones en niños con dentición mixta los que favorecerían un mejor diagnóstico de las anomalías dento-maxilofaciales. Objetivo: proponer el perfeccionamiento de las normas del cefalograma resumido de Ricketts para los niños. Materiales y Métodos: investigación observacional, descriptiva en cuatro poblaciones pertenecientes a las siguientes zonas del país: Matanzas, Cárdenas, Camagüey y Sancti Spíritus. El universo estuvo constituido por 1 657 niños de 9 años de edad. La muestra 163 niños, seleccionados por el método estratificado por conglomerados con oclusión dentaria normal. Se utilizó el análisis del cefalograma resumido de Ricketts y la prueba t de media para determinar los valores de las variables estudiadas y se hicieron estimaciones puntuales y por intervalos de confianza. Resultados: tres variables mostraron medias iguales a las normas de Ricketts: eje facial (90°), profundidad facial (87°) y profundidad maxilar (90°). En las restantes fueron comprobadas diferencias significativas. Se obtuvo un intervalo de confianza para cada una de las variables del cefalograma resumido de Ricketts. Conclusiones: solamente tres variables presentaron valores similares a los planteados por Ricketts, las restantes presentaron valores que difieren significativamente de los planteados por Ricketts. Se propone utilizar el intervalo de confianza obtenido en esta investigación en las variables del cefalograma resumido de Ricketts para diagnosticar las anomalías dento-maxilofaciales en niños cubanos (AU).

Introduction: in cephalometric findings from different countries in populations with normal growing and occlusion patterns, significant statistic differences are reported in relation to the values given in the cephalograms of Ricketts, Steiner, Downs, Jarabak, and others. In Cuba there are not studies of these patterns in children with mixed dentition that might allow a better diagnosis of dental, maxilla-facial anomalies. Objective: to propose the improvement of the standards of the Ricketts abridge cephalogram for children. Materials and methods: descriptive, observational research in four populations belonging to different regions of the country: Matanzas, Cárdenas, Camagüey and Sancti Spíritus. The universe was formed by 1675 children aged 9 years old; the sample, 163 children with normal dental occlusion, was chosen by the stratified method per conglomerate. The analysis of the Ricketts summarized cephalogram and the mean T test were used to determine the values of the studied variables; punctual estimates and estimates by confidence interval were carried out. Results: three variables showed equal means than Ricketts' standards: facial axis (90o), facial depth (87o) and maxillary depth (90o). In the remaining variables there were found significant differences. A confidence interval was obtained for every one of the Ricketts' summarized cephalogram variable. Conclusions: only three variables showed values similar to those given by Ricketts. The remaining ones presented values significantly differing from those established by Ricketts. The proposition is using the confidence interval obtained in this research in the variables of the Ricketts' summarized cephalogram to diagnose the dental-maxillafacial anomalies in Cuban children (AU).

Analysis of the stomatognathic system of children according orthodontic treatment needs.

PURPOSE: The present study evaluated electromyographic activity (EMG), masticatory performance, and tongue strength in children without and with orthodontic treatment needs. PATIENTS AND METHODS: A total of 90 children were screened and divided into the following groups: Group I (no treatment needed; mean age: 8.00 ± 0.43 years; n = 26), Group II (few malocclusions, treatment needed; mean age: 8.89 ± 0.43 years; n = 28), and Group III (slight-to-borderline treatment needed; mean age: 8.44 ± 0.22 years; n = 36). Orthodontic treatment need was classified on the basis of IOTN-DHC (Index of Orthodontic Treatment Need - Dental Health Component). The electromyographic Trigno EMG Systems was used for muscle analysis and the Iowa Oral Pressure Instrument (IOPI) was used to measure tongue strength. Data were analyzed using normality tests and one-way analysis of variance with a Bonferroni post hoc test (p ≤ 0.05). RESULTS: EMG in almost all mandibular movements was higher in Group III with statistically significant differences compared to position at rest: right masseter (p = 0.03); protrusion: left temporal (p = 0.02); saliva swallowing: left temporal (p = 0.05) and water swallowing: orbicularis oris mouth, right upper segment (p = 0.05). Lower masticatory performance was found in Group III, but the difference compared to Group I and II was not significant. There were no significant differences between the groups in terms of tongue strength. CONCLUSIONS: Children with borderline orthodontic treatment needs show functional disorders of the stomatognathic system.

Evaluation of stomatognathic problems in children with osteogenesis imperfecta (osteogenesis imperfecta - oi) - preliminary study.

According to epidemiological data, muscular dysfunctions of the masticatory system occur in 15-23% of the population. Preventive examinations of functional disorders of the stomatognathic system are, therefore, of particular importance. A distinct group of patients exposed to dysfunctions in the area of the masticatory organ locomotor apparatus comprises those with genetic diseases characterised by disorders in collagen formation. One of such diseases is osteogenesis imperfecta (OI) and dentinogenesis imperfecta that usually goes together with the former. AIM: The objective of this work was to evaluate the frequency with which particular disorders of the masticatory organ locomotor apparatus occur within the group of patients with osteogenesis imperfecta. MATERIAL AND METHODS: The study was performed on patients of the Orthopaedic Clinic of the Polish-American Paediatric Institute in Kraków. The mean age of the children was 7.9 years. In all the cases, a genetic diagnosis of OI has been confirmed. The research methods were based on an in-depth interview on family diseases, pregnancy, postnatal period, feeding, subjective assessment of dysfunctions in the stomatognathic system. An examination of the deformations in the stomatognathic system and the skeleton was conducted, as well as an examination of the trauma and tone of the jaw. The relationship between breastfeeding and swallowing and speech disorders was also evaluated. The impact of intubation on mandibular ranges was investigated. RESULTS: The results obtained were subjected to statistical analysis on the basis of which conclusions were drawn concerning disorders in the stomatognathic system which tend to occur in children with OI. The renunciation of breastfeeding significantly contributes to sucking and swallowing disorders, rumen disorders, as well as biomechanical disorders in the temporomandibular joint. A significant dependence between breastfeeding and swallowing problems was found, whereas there was no such dependence with respect to speech impediments. CONCLUSIONS: The results of the research conducted led to the following conclusions: 1. Among pediatric patients with OI there are disorders in the stomatognathic system. The most common dysfunctions are: abdominal, swallowing and sucking disorders, abnormal muscle structure of the rumen and biomechanical disorders in the temporomandibular joints. Breastfeeding significantly contributes to swallowing disorders. 2. The therapeutic process involving children with OI requires the cooperation of specialists in orthopedics, pediatrics, physiotherapy, orthodontics and neurologopedics to carry out comprehensive diagnostics and treatment tailored to the individual needs of the patient. 3. In order to draw final conclusions, there is a need for more research by means of objective tools, such as EMG and a condensate recorder.

Pfeiffer syndrome: oral healthcare management and description of new dental findings in a craniosynostosis.

Pfeiffer syndrome is a rare fibroblast growth factor receptor-related craniosynostosis with variable clinical presentations. We describe new dental findings of hypodontia, microdontia, dilacerations, and radicular dentin dysplasia in a 19-year-old girl, and discuss the oral health management.

Computational and clinical investigation on the role of mechanical vibration on orthodontic tooth movement.

The aim of this study is to investigate the biomechanics for orthodontic tooth movement (OTM) subjected to concurrent single-tooth vibration (50Hz) with conventional orthodontic force application, via a clinical study and computational simulation. Thirteen patients were recruited in the clinical study, which involved distal retraction of maxillary canines with 1.5N (150g) force for 12weeks. In a split mouth study, vibration and non-vibration sides were randomly assigned to each subject. Vibration of 50Hz, of approximately 0.2N (20g) of magnitude, was applied on the buccal surface of maxillary canine for the vibration group. A mode-based steady-state dynamic finite element analysis (FEA) was conducted based on an anatomically detailed model, complying with the clinical protocol. Both the amounts of space closure and canine distalization of the vibration group were significantly higher than those of the control group, as measured intra-orally or on models (p<0.05). Therefore it is indicated that a 50Hz and 20g single-tooth vibration can accelerate maxillary canine retraction. The volume-average hydrostatic stress (VHS) in the periodontal ligament (PDL) was computationally calculated to be higher with vibration compared with the control group for maxillary teeth and for both linguo-buccal and mesial-distal directions. An increase in vibratory frequency further amplified the PDL response before reaching a local natural frequency. An amplification of PDL response was also shown to be induced by vibration based on computational simulation. The vibration-enhanced OTM can be described by mild, vigorous and diminishing zones among which the mild zone is considered to be clinically beneficial.

Single stage correction of bilateral tessier 4 cleft.

Tessier facial clefts are among the rarest facial clefts reported in literature and many contradicting issues have always been rising over the management and surgical approaches involved during the craniofacial cleft repair. Among the craniofacial clefts Tessier no. 4 is an extremely rare facial anomaly, and there are very few evidence which clearly describe the surgical approaches and techniques. Often these type of craniofacial clefts yield very poor surgical results, and they require multidisciplinary sequential corrective surgeries. This article presents a rare case of an 18-month-old baby with bilateral Tessier no. 4 clefts and its successful rehabilitation.

Steinert syndrome and repercussions in dental medicine.

Steinert syndrome, also called myotonic dystrophy type 1, is a genetic disorder with autosomal dominant transmission characterized by myotonia and a multisystemic clinical picture that affects several tissues of the human body. The most common systemic phenotypes are: muscular, cardiac, respiratory, CNS, ocular, gynecological, digestive, orthopedical, as well as cognitive and psychological symptoms (cognitive decline). Muscles involved in voluntary movement are highly affected by myotonia especially distal muscles of upper limbs. These patients also show changes in face, chewing and pharynx muscles that can lead to swallowing and speech problems, dysphagia and in most cases to food aspiration and suffocation. Poor oral hygiene resulting from reduced motor mobility and reduced saliva flux can lead to gingival inflammation and periodontal disease. Other oral manifestations include disturbances at the temporomandibular articulation, dental occlusion changes and reduction in teeth number as a result of caries. Main causes of death are pneumonia and cardiac arrhythmias. The etiopathogeny of this syndrome is still not clear, conditioning the existence of a specific treatment for this disease. Nowadays, treatments consist on the release of the existing symptoms, in an attempt to give a better life quality to patients. It is very important to implement actions that can prevent complications and consequently decrease death. Treatments should be applied in an early stage of the disease. Bronchoscopy and artificial respiration should be used to prevent pneumonia, and regular electrocardiographic monitoring should be done to evaluate defects in the conductive system. Several approaches have been applied to rehabilitate swallowing dysfunction and avoid aspiration like videofluoroscopy, postural techniques and adjustment of diet type. It is the aim of this paper to clarify the ethiology, diagnosis, systemic and oral characteristics of the syndrome, as well as to discuss treatments to be applied according to patients affected organs.

Preenchimentos estéticos na cirurgia ortognática: há indicações? / Aesthetic augmentation in orthognathic surgery: are there indications?

Introdução: resultados estéticos na cirurgia ortognática são um dos principais objetivos dos pacientes, ortodontistas e cirurgiões. A correção da deformidade óssea maxilomandibular, geralmente, restabelece as funções do sistema estomatognático e a estética facial. No entanto, nem sempre obtém-se simetria e projeção desejada dos tecidos moles somente com a movimentação óssea. Nesse sentido, o preenchimento com o polimetilmetacrilato (PMMA) atua como coadjuvante na tentativa de se obter contornos faciais que alcancem as expectativas estéticas dos pacientes e profissionais. Objetivo: o presente artigo revisa a utilização do metilmetacrilato como material de preenchimento na região craniofacial, abordando a sua perspectiva histórica, vantagens e aspectos biológicos. As principais indicações são ilustradas com figuras e casos clínicos

Oral and craniofacial manifestations of Ellis-van Creveld syndrome: Case series.

PURPOSE: The objective of this case series was to determine the oral, dental and craniofacial features of patients with EvC syndrome. MATERIAL AND METHODS: Eight patients with EvC syndrome were enrolled. A complete family history, pedigree analysis, detailed medical history were collected. Findings of clinical examination, including craniofacial and orodental manifestations, and radiological investigations were thoroughly studied. RESULTS: All eight patients had characteristic face, hypertrophic frenulum, conical and peg-shaped teeth, hypodontia of deciduous and/or permanent teeth and also skeletal dysplasia, small chest, short stature and hypoplastic nails. Additionally dysmorphic filtrum, serrated appearance of gingiva, diastema, enamel hypoplasia, microdontia, taurodontism, single rooted permanent molar, delayed eruption and high caries rate were observed with varying degrees. Cephalometric evaluation revealed skeletal Class III growth pattern in four subjects and Class II growth pattern in one subject. CONCLUSION: Evaluation of craniofacial and orodental anomalies of EvC syndrome is required for accurate differential diagnosis from other congenital syndromes.

Efectos del tratamiento temprano en variables morfológicas, cinco años después de su aplicación / Effects of early treatment on morphological variables five years after application

Introducción: las pautas del tratamiento temprano propician una respuesta de desarrollo del sistema estomatognático. Objetivo: evaluar los efectos del tratamiento temprano en variables morfológicas, con técnicas de la rehabilitación neuro oclusal, cinco años después de ser aplicado durante la dentición temporal. Métodos: investigación de desarrollo, longitudinal y descriptiva, entre los años 2001 y 2010, en la Facultad de Estomatología de la Universidad de Ciencias Médicas de Villa Clara. Universo: 2 215 niños de cinco años de edad perteneciente a instituciones infantiles de la ciudad de Santa Clara (2001-2002). Se utilizó un método estratificado por etapas. Las variables de estudio se analizaron en tres etapas evolutivas: inicial, intermedia y final. La muestra fue de 59 niños con interferencias oclusales durante la dentición temporal que fueron tratados con terapias funcionales, durante el año 2002 y evaluados un año después (primera etapa). La segunda etapa se desarrollo durante los años 2007-2010; la muestra fue de 46 niños con diez años de edad. Se diseñó un modelo de consentimiento informado que estableció el compromiso de colaboración de los niños seleccionados. Se realizaron pruebas estadísticas: test de Friedman, Wilcoxon, Fisher y medida clásica de chi cuadrado. Resultados: se observó un aumento del resalte en 33 de los casos y predominó el escalón mesial (87 por ciento) al final del estudio. Se mantuvo la tendencia al aumento en la anchura intermolar temporal. Los valores medios a nivel de los primeros molares permanentes se comportaron similares a la norma descrita por Mayoral (47 mm). Conclusiones: los cambios favorables observados en las variables morfológicas objeto de estudio, cinco años después de ser aplicado el tratamiento temprano con técnicas de la rehabilitación neuro oclusal, corroboran que los cinco primeros años de la vida del niño constituye la etapa ideal para comenzar a tratar las alteraciones del sistema estomatognático(AU)

Introduction: early treatment typically obtains an adequate developmental response from the stomatognathic system. Objective: evaluate the effects of early treatment on morphological variables using neuro-occlusal rehabilitation techniques five years after application during primary dentition. Methods: adevelopmental longitudinal descriptive study was conducted from 2001 to 2010 at the School of Dentistry of the University of Medical Sciences of Villa Clara. The study universe was 2 215 five-year-old children from educational institutions of the city of Santa Clara (2001-2002). A staged stratified method was applied. Study variables were analyzed along three evolution stages: initial, intermediate and final. The study sample was composed of 59 children with occlusal interferences during primary dentition, which were treated with functional therapies in 2002 and evaluated one year later (first stage). The second stage extended from 2007 to 2010. The sample consisted of 46 ten-year-old children. An informed consent form was developed to record the commitment of the children selected with the study. The statistical tests performed were Friedman's, Wilcoxon's, Fisher's and classical chi-square measure. Results: increased overjet was found in 33 of the cases, with a predominance of the mesial step (87 percent) at the end of the study. A tendency to increased temporary intermolar width was observed throughout the study. Mean values for the first permanent molars were similar to the standard described by Mayoral (47 mm). Conclusions: the favorable changes found in the morphological variables under study five years after application of early treatment with neuro-occlusal rehabilitation confirm that the first five years in the life of a child are the ideal stage to start treating alterations of the stomatognathic system(AU)