Itinerário terapêutico de crianças com fissura labiopalatina / Itinerario terapéutico del niño con labio y paladar hendido / Therapeutic itinerary of children with cleft lip and palate

Resumo Objetivo conhecer o itinerário terapêutico de crianças com fissuras de lábio e/ou palato. Método estudo descritivo de natureza qualitativa realizado com famílias de crianças com fissura labiopalatina. A coleta de dados ocorreu entre junho e julho de 2022 por meio de entrevistas semiestruturadas, e aplicou-se a análise de conteúdo temática. Resultados participaram oito famílias e emergiram três categorias: I) Enfrentando o desconhecido: diagnóstico de fissura labiopalatina na criança - notaram-se sentimentos de choque, medo e incerteza que permeiam durante o diagnóstico precoce ou tardio; II) Um caminho árduo: pausas e recomeços durante o itinerário terapêutico - observou-se que as famílias percorreram caminhos diferentes devido à especificidade da configuração familiar e da própria fissura; III) Tecendo a rede de apoio para o cuidado - mostrou-se ser de extrema necessidade o apoio dos amigos, família, profissionais e instituições de saúde. Conclusões e implicações para prática o itinerário terapêutico de crianças com fissura labiopalatina é árduo e acompanhado de desafios, que se iniciam no diagnóstico e permanecem após o nascimento. Espera-se que os resultados deste estudo suscitem o diálogo entre os profissionais de saúde e se familiarizem com as necessidades dessa população e possam atuar nos diferentes pontos de atenção à saúde.

Resumen Objetivo conocer el itinerario terapéutico de los niños con labio hendido y/o paladar hendido. Método estudio cualitativo descriptivo realizado con familias de niños con labio y paladar hendido. La recolección de datos se realizó entre junio y julio de 2022 mediante entrevistas semiestructuradas, y se aplicó análisis de contenido temático. Resultados participaron ocho familias y surgieron tres categorías: I) Frente a lo desconocido: diagnóstico de labio y paladar hendido en niños: se observaron sentimientos de shock, miedo e incertidumbre que permean durante el diagnóstico temprano o tardío; II) Un camino arduo: pausas e inicios durante el itinerario terapéutico - se observó que las familias siguieron caminos diferentes debido a la especificidad de la configuración familiar y de la propia hendidura; III) Tejer la red de apoyo para la atención: el apoyo de amigos, familiares, profesionales e instituciones de salud resultó ser extremadamente necesario. Conclusiones e implicaciones para la práctica el itinerario terapéutico de los niños con labio y paladar hendido es arduo y está acompañado de desafíos, que comienzan desde el diagnóstico y continúan después del nacimiento. Se espera que los resultados de este estudio fomenten el diálogo entre los profesionales de la salud y conozcan las necesidades de esta población y puedan actuar en los diferentes puntos de la atención en salud.

Abstract Objective to understand the therapeutic itinerary of children with cleft lip and/or palate. Method a descriptive qualitative study carried out with families of children with cleft lip and palate. Data collection took place between June and July 2022 through semi-structured interviews, and thematic content analysis was applied. Results eight families participated and three categories emerged: I) Facing the unknown: diagnosis of cleft lip and palate in children - feelings of shock, fear and uncertainty were noted that permeate during early or late diagnosis; II) An arduous path: pauses and beginnings during the therapeutic itinerary - it was observed that the families followed different paths due to the specificity of the family configuration and the cleft itself; III) Weaving the support network for care - support from friends, family, professionals and health institutions proved to be extremely necessary. Conclusions and implications for practice the therapeutic itinerary of children with cleft lip and palate is arduous and followed by challenges, which begin at diagnosis and continue after birth. It is hoped that the results of this study will encourage dialogue among health professionals and become familiar with the needs of this population and be able to act at different points of health care.

Displasia frontonasal / Frontonasal dysplasia

Los trastornos del desarrollo son aquellos padecimientos que se manifiestan por defectos en la embriogénesis de la región afectada. La cara del ser humano comienza su formación alrededor de la cuarta semana de vida intrauterina y se manifiesta gracias a la fusión de cinco prominencias: dos pares conocidas como maxilar y mandibular, y una impar conocida como frontonasal. Cuando esta fusión no se lleva a cabo de una forma óptima, aparecen numerosas alteraciones del desarrollo como el labio y paladar hendido, y la displasia frontonasal. La displasia frontonasal produce frecuentemente afecciones oculares, nasales y orales. Dentro de las manifestaciones orales destacan una forma atípica de hendidura labial o palatina, afecciones dentales y alteraciones en el crecimiento de la cara. Dada la gran relación que este padecimiento tiene con la cavidad oral resulta importante que el odontólogo conozca la etiología y las características clínicas de este trastorno (AU)

Developmental disorders are those conditions that are manifested by defects in the embryogenesis of the affected region. The human face begins its formation around the fourth week of intrauterine life and is manifested thanks to the fusion of five prominences: two pairs known as maxillary and mandibular and odd one known as frontonasal. When this fusion is not carried out in an optimal way, numerous developmental alterations appear, such as cleft lip and palate and frontonasal dysplasia. Frontonasal dysplasia frequently produces ocular, nasal and oral affections. Among the oral manifestations, and atypical form of clef lip and/or palate, dental affections and alterations in the growth of the face stand out. Given the great relationship that this condition has with the oral cavity, it is important for the dentist to know the etiology and clinical characteristics of this disorder (AU)

Uso del modelador nasolabial (NAM) como tratamiento ortopédico prequirúrgico de paciente con labio y paladar hendido / Use of nasoalveolar molding (NAM) as a pre-surgical orthopedic treatment for patients with cleft lip and palate

Introducción: el labio y paladar hendido es la malformación craneofacial congénita más frecuente y es producida por una falla en la fusión de procesos faciales durante periodos iniciales en el desarrollo embrionario. Objetivo: el objetivo de la ortopedia prequirúrgica es facilitar la primera intervención para la corrección de esta condición de manera que disminuya el trauma quirúrgico, al obtener una menor formación de cicatrices y menor riesgo de complicaciones postquirúrgicas, además de ayudar a obtener una óptima deglución en el paciente para mejorar el aspecto nutricional en el bebé. Material y métodos: el paciente fue llevado a la clínica a las dos semanas de nacido para la toma de impresión y colocación de un aparato ortopédico; se le colocó el NAM (nasoalveolar molding), la cinta transport, además se realizaban citas una vez al mes para evaluar el stent nasal, activarlo y revisar que la colocación de la cinta estuviese correcta, así como para comprobar que la alimentación del bebé estuviera mejorando. Resultados: se observa una mejoría anatómica y fisiológica, también una disminución de la fisura labial de 6 mm y una remodelación nasal. Se notó una mejora en deglución y alimentación con ganancia de peso. Conclusiones: la ortopedia prequirúrgica con el NAM es una terapia aceptable y recomendable para los pacientes con labio y paladar hendido, ya que nos ayudan a generar mejores resultados quirúrgicos, además de colaborar con el aspecto nutricional del bebe (AU)

Introduction: cleft lip and palate is a craniofacial malformation most frequent congenital and is produced by a failure to fuse facial processes during early periods in embryonic development. Objective: the objective of pre-surgical orthopedics is to facilitate the first intervention for the correction of this condition, reducing surgical trauma, obtaining less scar formation and less risk of post-surgical complications, in addition to helping the patient obtain better swallowing to improve the nutritional aspect in the baby. Material and methods: the patient came to the clinic at two weeks of age for impression taking and placement of the orthopedic device. The NAM (nasoalveolar molding) and the transport tape were placed and appointments were made once a month to evaluate the nasal stent, activate it and check that the placement of the tape was correct, as well as to check that the baby's feeding was improving. Results: an anatomical and physiological improvement is observed, as well as a 6mm decrease in the cleft lip and nasal remodeling. An improvement in swallowing and feeding with weight gain was observed. Conclusions: pre-surgical orthopedics with NAM is an acceptable and recommended therapy for patients with cleft lip and palate since it helps us to generate better surgical results in addition to collaborating with the nutritional aspect of the baby (AU)

Investigación en fisuras faciales: quo vadis España? / Investigation in facial fissures: quo vadis Spain?

Las fisuras orofaciales representan un grupo heterogéneo de malformaciones congénitas que afectan a distintas estructuras de la cavidad oral y de la cara. Globalmente, los bebés con estos trastornos presentan una mayor morbilidad y mortalidad a lo largo de su vida en comparación con individuos no afectados. Por ello, los avances en la investigación biomédica resultan ineludibles. Así, el objetivo general de este trabajo fue llevar a cabo una revisión bibliográfica para analizar narrativamente los 10 principales estudios primarios sobre fisuras orofaciales llevados a cabo en España, publicados del 2018 hasta la actualidad. Según esto, a nivel institucional, destaca la Universidad Complutense de Madrid (UCM) con cuatro artículos publicados por el grupo de investigación UCM 920202. También sobresale la Universidad Rey Juan Carlos de Madrid, con tres artículos relacionados con diferentes aspectos de la personalidad y la calidad de vida de los pacientes fisurados, así como otras muchas variables cognitivo-emocionales. En relación con la Universidad de Valencia, encontramos dos artículos llevados a cabo en amplias muestras de pacientes con fisuras. Por último, en Barcelona resulta destacable un estudio observacional sobre problemas otorrinolaringológicos en pacientes operados de fisura palatina. En conclusión, si bien en los últimos años se han publicado varios artículos sobre distintos aspectos relacionados con las fisuras, aún queda mucho trabajo por hacer. España debería seguir potenciando proyectos con líneas de trabajo centradas en estas alteraciones del desarrollo craneofacial. Se necesitan estudios amplios, multicéntricos y colaborativos, para ahondar en los mecanismos etiológicos y, en última instancia, en las posibles herramientas para su prevención. Del mismo modo, se necesitan ayudas para dilucidar mejor las cuestiones relacionadas con los tratamientos en todas las dimensiones de la salud, preferentemente a partir de ensayos clínicos controlados aleatorizados, que faciliten la traslación de conocimientos y su accesibilidad universal dentro del sistema sanitario público español.

SUMMARY: Orofacial clefts represent a heterogeneous group of congenital malformations affecting different structures of the oral cavity and face. Overall, infants with these disorders have a higher lifetime morbidity and mortality compared to unaffected individuals. Therefore, advances in biomedical research are unavoidable. Thus, the overall objective of this work was to conduct a literature review to narratively analyse the 10 main primary studies on orofacial clefts carried out in Spain, published from 2018 to date. According to this review, at an institutional level, the Complutense University of Madrid (UCM) is notable with 4 articles published by the UCM 920202 research group. The Rey Juan Carlos University of Madrid also stands out, with three papers related to different aspects of the personality and quality of life of cleft patients, as well as many other cognitive-emotional variables. In relation to the University of Valencia, we found two studies carried out on large samples of cleft patients. Finally, in Barcelona, an observational study on otorhinolaryngological problems in cleft palate patients is noteworthy. In conclusion, although several studies have been published in recent years on different aspects related to clefts, there is still much work to be done. Spain should craniofacial development. Large, multicenter and collaborative studies are needed to delve deeper into the aetiological mechanisms and, ultimately, into the possible tools for their prevention. Similarly, support is needed to better elucidate questions related to treatments in all dimensions of health, preferably randomised controlled clinical trials, which facilitate the transfer of knowledge and its universal accessibility within the Spanish public health system.

Effectiveness of iliac cancellous bone grafting in alveolar cleft repair and analysis of factors affecting it / 华西口腔医学杂志

OBJECTIVES@#To review the effectiveness of secondary alveolar bone grafting using iliac cancellous bone in patients with unilateral complete alveolar cleft and to investigate the factors influencing it.@*METHODS@#A retrospective study of 160 patients with unilateral complete alveolar clefts who underwent iliac cancellous bone graft repair at the Department of Cleft Lip and Palate Surgery, West China Hospital of Stomatology, Sichuan University, was conducted. Eighty patients in the young age group (6-12 years) and 80 in the old age group (≥13 years) were included. Bone bridge formation was determined using Mimics software, and the volume was measured to calculate the iliac implantation rate, residual bone filling rate, and resorption rate. The factors that affected bone grafting in both subgroups were investigated.@*RESULTS@#Using bone bridge formation as the clinical success criterion, the success rate for the entire population was 71.25%, with a significant difference of 78.75% and 63.75% for the young and old age groups, respectively (P=0.036). The gap volume in the latter was significantly larger than that in the former (P<0.001). The factors that influenced bone grafting in the young group were the palatal bone wall (P=0.006) and history of cleft palate surgery (P=0.012), but only the palatal bone wall affected the outcome in the old age group (P=0.036).@*CONCLUSIONS@#The results of alveolar bone grafting for the old age group were worse than those for the young age group. The palatal bone wall was an important factor that affected alveolar bone grafting, and alveolar bone grafting in the young patients was influenced by the history of cleft palate surgery.

Two-stage alveolar bone grafting for nasal floor reconstruction in adult cleft patients / 华西口腔医学杂志

Alveolar cleft is one of the key links of cleft lip and palate reconstruction due to its close relationship with tooth and jaw coordination and nasolabial deformity. The alveolar bone graft repairs the hole in the gum ridge and stabilizes the bone arch, providing better support for the base of the nose and new bone for the roots of the developing teeth to grow into. Unfortunately, bone graft failure in the traditional way, even among minor clefts, bony hypoplasia, or absence that affects the nasal base and piriform rim, is common. Two-stage alveolar bone grafting, which has advantages in addressing the underlying skeleton and deficiency, could be an optional surgical procedure for nasal floor reconstruction in adult patients with a broad alveolar cleft.

Study on brain mechanism of rehabilitation training of articulation disorder in cleft lip and palate patients based on functional magnetic resonance imaging / 生物医学工程学杂志

The cleft lip and palate (CLP) is one of the most common craniofacial malformations in humans. We collected functional magnetic resonance data of 23 CLP patients before rehabilitation training (Bclp) and 23 CLP patients after rehabilitation training (Aclp), who were performing Chinese character pronunciation tasks, and performed brain activation analysis to explore the changes of brain mechanism in CLP patients after articulation disorder rehabilitation training. The study found that Aclp group had significant activation in the motor cortex, Broca area, Wernicke area and cerebellum. While the Bclp group had weak activation in the motor cortex with a small activation range. By comparing the differences and co-activated brain regions between the two groups, we found that rehabilitation training increased the activity level of negatively activated brain areas (cerebellum, left motor area, Wernicke area, etc.) to a positive level. At the same time, the activity level of weakly activated brain areas (right motor area, Broca area, etc.) was also increased. Rehabilitation training promoted the activity level of articulation-related brain regions. So that the activation intensity of articulation-related brain regions can be used as a quantifiable objective evaluation index to evaluate the effect of rehabilitation training, which is of great significance for the formulation of rehabilitation training programs.

Progress in research of risk prediction of non-syndromic oral clefts using genetic information / 中华流行病学杂志

Non-syndromic oral cleft (NSOC), a common birth defect, remains to be a critical public health problem in China. In the context of adjustment of childbearing policy for two times in China and the increase of pregnancy at older childbearing age, NSOC risk prediction will provide evidence for high-risk population identification and prenatal counseling. Genome-wide association study and second generation sequencing have identified multiple loci associated with NSOC, facilitating the development of genetic risk prediction of NSOC. Despite the marked progress, risk prediction models of NSOC still faces multiple challenges. This paper summarizes the recent progress in research of NSOC risk prediction models based on the results of extensive literature retrieval to provide some insights for the model development regarding research design, variable selection, model-build strategy and evaluation methods.

The Tip60/Ep400 chromatin remodeling complex impacts basic cellular functions in cranial neural crest-derived tissue during early orofacial development / 国际口腔科学杂志·英文版

The cranial neural crest plays a fundamental role in orofacial development and morphogenesis. Accordingly, mutations with impact on the cranial neural crest and its development lead to orofacial malformations such as cleft lip and palate. As a pluripotent and dynamic cell population, the cranial neural crest undergoes vast transcriptional and epigenomic alterations throughout the formation of facial structures pointing to an essential role of factors regulating chromatin state or transcription levels. Using CRISPR/Cas9-guided genome editing and conditional mutagenesis in the mouse, we here show that inactivation of Kat5 or Ep400 as the two essential enzymatic subunits of the Tip60/Ep400 chromatin remodeling complex severely affects carbohydrate and amino acid metabolism in cranial neural crest cells. The resulting decrease in protein synthesis, proliferation and survival leads to a drastic reduction of cranial neural crest cells early in fetal development and a loss of most facial structures in the absence of either protein. Following heterozygous loss of Kat5 in neural crest cells palatogenesis was impaired. These findings point to a decisive role of the Tip60/Ep400 chromatin remodeling complex in facial morphogenesis and lead us to conclude that the orofacial clefting observed in patients with heterozygous KAT5 missense mutations is at least in part due to disturbances in the cranial neural crest.

Differences of temporomandibular joint symmetry in patients with unilateral complete cleft lip and palate at different developmental stages / 中南大学学报(医学版)

OBJECTIVES@#Currently, the research results regarding the bilateral temporomandibular joint symmetry in patients at different ages with unilateral complete cleft lip and palate (UCLP) are still controversial. In this study, the position of condyle in the articular fossa and morphology of condyle in UCLP patients at different developmental stages was measured and analyzed to explore the asymmetry difference, which can provide a new theoretical basis for the sequential therapy.@*METHODS@#A total of 90 patients with UCLP were divided into a mixed dentition group (31 cases), a young permanent dentition group (31 cases) and an old permanent dentition group (28 cases) according to age and dentition development. Cone beam computed tomography (CBCT) images were imported into Invivo5 software for 3D reconstruction, and the joint space, anteroposterior diameter, medio-lateral diameter, and height of condylar were measured, and its asymmetry index was calculated.@*RESULTS@#The asymmetry index of condylar height and anteroposterior diameter among the 3 groups, from small to large, was the mixed dentition group<the young permanent dentition group<the old permanent dentition group (both P<0.05). There was no significant difference in condylar anteroposterior diameter and asymmetry index between the mixed dentition group and the young permanent dentition group (both P>0.05), all of them were lower than those in the old permanent dentition group (both P<0.05). Compared with the normal side, the height of fracture condyle was smaller among the 3 groups (all P<0.05), and the anterior joint space was smaller (P<0.05) and the posterior joint space was larger (P<0.05) in the mixed dentition group.@*CONCLUSIONS@#In patients with UCLP, the asymmetry of condylar morphology increases with age, but the condylar position tends to normal. These results suggest that early treatment has important clinical significance for the morphologic development of temporomandibular joint in UCLP patients.

Vírus e fissuras orofaciais: um histórico isolado e de associação / Viruses and orofacial clefts: an isolated and associational history / Virus y fisuras orofaciales: una historia aislada y asociativa

Os vírus são microrganismos comumente associados as doenças e infectam todos os seres vivos. Atuam de forma direta e indireta levando a pressão seletiva, com papel significativo e ainda em exploração no planeta. As fissuras orofaciais são anomalias congênitas de etiologia complexa e multifatorial, sendo as infecções virais durante a gestação um dos possíveis fatores etiológicos. A história da humanidade frente aos vírus e fissuras orofaciais de forma isolada é vasta, remontando a períodos antes de Cristo, seja por meio de leis para o controle de pragas e/ou por lendas de míticas criaturas deificadas e/ou demonizadas, cuja criação está fundamentada na Teoria Alegórica do surgimento das mitologias, demonstrando assim o interesse do ser humano e sua curiosidade em inovação e explicação destes assuntos. Considerando a relevância histórica, bem como a possível relação etiológica destes dois elementos, uma revisão da literatura foi realizada para apresentar a história mitológica e científica dos vírus e fissuras orofaciais, de forma isolada e associadas para fins de comparação. Para isso, foram utilizadas as bases PubMed/Medline, SciElo, LILACS e Portal Periódicos (CAPES) com os descritores: Virus, Anomalias/Anomalies, Virus and Anomalias/Virus and Anomalies, A History of viruses/História dos vírus, Virus and History/História and Virus, Virus and Myth/Virus and Mito, Anomalias and Mitos/Anomalies and Myths, Vampires and Virus/Vampiros and Virus. Enquanto o histórico mitológico é cheio de teorias contraditórias, o histórico cientifico acadêmico se revela coerente, porém resistente as novas áreas de atuação, não ponderando novas possibilidades e limitando a exploração científica, que só pôde ser alcançada nos séculos atuais. Quanto a associação, a linha de pesquisa relacionando vírus e fissuras orofaciais não possui nem meio século de existência, propiciando um grande campo a ser explorado e na mesma medida limitando os benefícios em prevenção que poderiam ser obtidos através destes estudos.

Viruses are microorganisms commonly associated with diseases that infect all living beings, they act directly and indirectly leading to selective pressure, their role on the planet is significant and still under exploration. Orofacial clefts are congenital anomalies that have a complex multifactorial etiology, with viral infections during pregnancy being one of the possible etiological factors. The history of humanity in the face of viruses and orofacial clefts in isolation is vast, dating back to periods before Christ, whether through laws for pest control and/or legends of mythical deified and/or demonized creatures, whose creation is fundamentalized in the Allegorical Theory of the emergence of mythologies, thus demonstrating the interest of human beings and their curiosity in innovation and explanation of these subjects. Considering the historical relevance, as well as the possible etiology relationship of these two elements, we carried out a literature review to present the mythological and scientific history of viruses and orofacial clefts, isolated and associated for comparison purposes. For this intent, the bases PubMed/Medline, SciElo, LILACS and Portal Periódicos (CAPES) were selected with the descriptors: A History of viruses/História dos vírus, Virus and History/História and Virus, Virus and Myth/Virus and Mito, Anomalias and Mitos/Anomalies and Myths, Vampires and Virus/Vampiros and Virus. While the mythological history is full of contradictory theories, the academic, scientific history proves to be consistent, but resistant to new areas of action, not considering new possibilities and limiting scientific exploration, which can only be achieved in the present centuries. As for the association, the line of research relating viruses and orofacial clefts does not even have half a century of existence, providing a large field to be explored and at the same time limiting the benefits of prevention that could be obtained through these studies.

Los virus son microorganismos comúnmente asociados a enfermedades que infectan a todos los seres vivos, actúan directa e indirectamente provocando presión selectiva, su papel en el planeta es significativo y aún en exploración. Las hendiduras orofaciales son anomalías congénitas que tienen una compleja etiología multifactorial, siendo las infecciones virales durante el embarazo uno de los posibles factores etiológicos. La historia de la humanidad frente a los virus y las hendiduras orofaciales de forma aislada es vasta, remontándose a períodos anteriores a Cristo, ya sea a través de leyes para el control de plagas y/o leyendas de criaturas míticas deificadas y/o demonizadas, cuya creación se fundamentaliza en la Teoría Alegórica del surgimiento de las mitologías, demostrando así el interés del ser humano y su curiosidad en la innovación y explicación de estos temas. Considerando la relevancia histórica, así como la posible relación etiológica de estos dos elementos, realizamos una revisión bibliográfica para presentar la historia mitológica y científica de los virus y las hendiduras orofaciales, aislados y asociados para fines de comparación. Para ello, se seleccionaron las bases PubMed/Medline, SciElo, LILACS y Portal Periódicos (CAPES) con los descriptores: A History of viruses/História dos vírus, Virus and History/História and Virus, Virus and Myth/Virus and Mito, Anomalias and Mitos/Anomalías y Mitos, Vampiros and Virus/Vampiros y Virus. Mientras que la historia mitológica está llena de teorías contradictorias, la historia académica, científica, se muestra coherente, pero resistente a nuevos campos de actuación, no considerando nuevas posibilidades y limitando la exploración científica, que sólo puede alcanzarse en los siglos actuales. En cuanto a la asociación, la línea de investigación que relaciona virus y hendiduras orofaciales no tiene ni medio siglo de existencia, proporcionando un gran campo a ser explorado y al mismo tiempo limitando los beneficios de prevención que podrían ser obtenidos a través de estos estudios.

Does the rehabilitation treatment predispose the patient with cleft to endodontic treatment?

Aim: to verify the relation between endodontic treatment of teeth adjacent to the cleft area and the rehabilitation of patients with unilateral cleft lip and palate, at the Hospital for Rehabilitation of Craniofacial Anomalies. Methods: the present split-mouth study was composed of 406 individuals with complete unilateral cleft lip and palate who had completed the rehabilitation process in a single tertiary cleft center (55.9% males). The information was collected from the dental history on the patients' records and radiographs. The frequency of endodontic treatment in the upper incisors and canines was calculated for the cleft and non-cleft sides. The comparison between sides was performed by the chi-square test (p <0.05). Results: endodontic treatment was more frequent in teeth adjacent to the cleft than in contralateral teeth. The frequency of endodontic treatment in at least one tooth adjacent to the cleft was 18.97%, and 11.6% on the contralateral side. It was observed that endodontic treatment was necessary in 63.5% of patients who had been submitted to orthodontic treatment, 42.4% of those rehabilitated with fixed partial dentures and 12.0% of patients who underwent dental reshaping of teeth adjacent to the cleft. The treatments performed included vital pulp therapy (46.1%), non-vital pulp therapy (46.8%) and endodontic retreatment (7.1%). Conclusion: in individuals with complete cleft lip and palate, teeth close to the bone defect area and used for rehabilitation treatment presented greater need of endodontic intervention

Higher maternal age is associated with higher occurrence of cleft lip/palate in neonates under intensive care

Aim: To assess the prevalence of cleft lip and/or cleft palate (CL/P) and associated variables in neonates admitted to neonatal intensive care units (ICU). Methods: Medical charts for neonates born and admitted to the ICU between 2012 and 2018 were reviewed. Obstetric and neonatal variables were collected by a trained researcher. In the case group, all neonates with CL/P were included. The control group was formed by matching sex, prematurity and month of birth using random number generation. Neonates with congenital malformations were excluded from the control group. Adjusted logistic regression was used (p<0.05). Results: The prevalence of CL/P was 0.43% (n=15). Five cases were excluded, as pairing was not possible. Twenty neonates were included in the control group. In the final multivariate model, CL/P was only associated with increased maternal age. For each year of increase in maternal age, neonates had a 35.2% higher chance of presenting CL/P (95% confidence interval: 1.021­1.792). Conclusions: Higher maternal age was associated with higher occurrence of CL/P in neonates admitted to the ICU. No other neonatal or maternal independent variables were associated with CL/P. Due to missing data, interpretation of study results must be approached with caution

Surgical effects of rehabilitation protocols on dental arch occlusion of children with cleft lip and palate

Aim: to evaluate the surgical effects of two rehabilitation protocols on dental arch occlusion of 5-year-old children with or without cleft lip and palate. Methods: this is a retrospective longitudinal study the sample comprised 45 digitized dental casts divided into followed groups: Group 1 (G1) ­ children who underwent to cheiloplasty (Millard technique) at 3 months and to one-stage palatoplasty (von Langenbeck technique) at 12 months; Group 2 (G2) ­ children who underwent to cheiloplasty (Millard technique) and two-stage palatoplasty (Hans Pichler technique for hard palate closure) at 3 months and at 12 months to soft palate closure (Sommerlad technique); and Group 3 (G3) ­ children without craniofacial anomalies. Linear measurements, area, and occlusion were evaluated by stereophotogrammetry software. Shapiro-Wilk test was used to verify normality. ANOVA followed by posthoc Tukey test and Kruskal-Wallis followed by posthoc Dunn tests were used to compared groups. Results: For the measures intercanine distance (C-C'), anterior length of dental arch (I-CC'), and total length of the dental arch (I­MM'), there were statistical differences between G1x G3 and G2xG3, the mean was smaller for G1 and G2. No statistically significant differences occurred in the intermolar distance and in the dental arch area among groups. The occlusion analysis revealed significant difference in the comparison of the three groups (p=0.0004). Conclusion: The surgical effects of two rehabilitation protocols affected the occlusion and the development of the anterior region of the maxilla of children with oral clefts when compared to children without oral clefts.

Fisura labio palatina: revisión de la literatura / Cleft lip and palate: literature review

La formación del paladar ocurre entre la quinta y undécima semana de vida intrauterina producto de la unión del paladar primario y secundario. Por otra parte, la formación del labio superior ocurre entre la quinta y sexta semana del desarrollo, y se configura en su parte media por la fusión de los procesos nasales mediales y lateralmente, a expensas de los procesos maxilares. La prevalencia de las fisuras labiales y/o fisura palatina varía según las distintas etnias, con cifras entre 0,7 hasta 1,1 casos por 1000 nacidos vivos. El objetivo de este trabajo fue realizar una revisión bibliográfica sobre aspectos epidemiológicos, mecanismos genéticos moleculares y ambientales que influyen en la ocurrencia de la fisura labial, fisura palatina y fisura labio palatina. La búsqueda bibliográfica se realizó en las bases de datos PUBMED, SCOPUS, SPRINGER, SCIENCEDIRECT utilizando los términos en inglés "cleft lip and palate", "cleft lip", "cleft palate" y "embriology". Entre los criterios de inclusión se consideraron estudios realizados en humanos y animales, publicados entre los años 2015 y 2021. La búsqueda arrojó un total de 407 trabajos, de los cuales tras un filtro por título y resumen quedaron un total de 38 artículos, en los cuales se realizó un análisis de texto completo para finalmente seleccionar 26 artículos que abarcan temas genéticos-moleculares, ambientales, epidemiológicos y sindrómicos. Además se incorporaron por búsqueda manual, 6 documentos asociados a libros de texto, y artículos científicos, sin considerar el criterio inclusión de tiempo. Dentro de esta revisión se describe la fuerte asociación entre las fisuras orales y las mutaciones de genes Msx1, sonic hedgehog, proteínas morfogenéticas óseas y factor de crecimiento fibroblástico durante la migración de las células de la cresta neural y la modelación y formación del paladar. La ausencia de ácido fólico durante el desarrollo del paladar y la presencia de hipoxia por exposición a humo, son los factores ambientales observados con mayor frecuencia en malformaciones orofaciales.

SUMMARY: Palate formation occurs between the fifth and eleventh week of intrauterine life as a result of the union of the primary and secondary palate. On the other hand, the formation of the upper lip occurs between the fifth and sixth week of development, and is configured in its middle part by the fusion of the medial and lateral nasal processes, at the expense of the maxillary processes. The prevalence of cleft lips and / or cleft palate varies according to the different ethnic groups, with figures ranging from 0.7 to 1.1 cases per 1000 live births. The aim of this work was to carry out a literature review on epidemiological aspects, molecular and environmental genetic mechanisms that influence the occurrence of cleft lip, cleft palate and its embriology. The literature search was carried out in the databases PUBMED, SCOPUS, SPRINGER, SCIENCEDIRECT using the English terms "cleft lip and palate", "cleft lip", "cleft palate" and "embryology". Inclusion criteria included studies carried out in humans and animals, published between 2015 and 2021. The search yielded a total of 407 works, of which after a filter by title and abstract, a total of 38 articles remained, in which a text analysis was carried out complete to finally select 26 articles that cover genetic-molecular, environmental, epidemiological and syndromic topics. In addition, 6 documents associated with textbooks and scientific articles were incorporated by manual search, without considering the inclusion criterion of time. This review describes the strong association between oral fissures and mutations of genes Msx1, sonic hedgehog, bone morphogenetic proteins and fibroblast growth factor during migration of neural crest cells and palate shaping and formation. Lack of folic acid during palae development dar and the presence of hypoxia due to exposure to smoke, are the environmental factors most frequently observed in orofacial malformations.

Dental treatment before surgery in a patient with cleft lip and palate: case report / Efeito do Cissus quadrangularis na cicatrização de fraturas em animais de laboratório

Introduction: orofacial clefts are common congenital malformations with an important social, psychological, and economic impact. The treatment of this condition may include different surgical procedures that previously require an adequate oral condition. Case report: we report a case of dental treatment before palatoplasty in a male patient of 17 years-old with bilateral cleft lip and palate and lesion in the jugal mucosa. Final considerations: the adequacy of the oral environment is a fundamental step in the treatment of patients with orofacial clefts and aims to restore oral health regardless of the degree of complexity of the dental treatment through the reduction of the pathogenic microbiota, elimination of retentive niches, instructions on diet and adequate oral hygiene and constant patient motivation.

Objetivos: as fissuras orofaciais são malformações congênitas comuns, com importante impacto social, psicológico e econômico. O tratamento dessa condição pode incluir vários procedimentos cirúrgicos que requerem previamente uma adequada condição bucal. Relato de caso: Relatamos o caso de tratamento odontológico prévio a palatoplastia em um paciente do gênero masculino de 17 anos de idade, com fissura transforame incisivo bilateral e lesão em mucosa jugal. Considerações finais: A adequação do meio bucal é uma etapa fundamental no tratamento de pacientes com fissuras orofaciais e visa restabelecer a saúde bucal independente do grau de complexidade do tratamento odontológico por meio da redução da microbiota patogênica, eliminação de nichos retentivos, orientações sobre dieta e higiene bucal adequada e motivação constante do paciente.

Modificación de Mulliken en la reparación de la fisura labial unilateral incompleta en Matanzas, 2016-2018 / Mulliken modification in the repair of incomplete unilateral cleft lip in Matanzas, 2016-2018

RESUMEN El impacto biológico, social, económico y psicológico que acarrea la fisura labial conlleva a aplicar nuevas modificaciones en la corrección quirúrgica de sus técnicas de tratamiento. El objetivo de esta investigación fue evaluar los resultados de la técnica quirúrgica de Rotación y Avance de Millard modificada por Mulliken en la corrección de la fisura labial unilateral incompleta en Matanzas. Se realizó un estudio de casos, longitudinal, prospectivo en el Hospital Docente Pediátrico «Eliseo Noel Caamaño¼ de Matanzas, entre 2016 y 2018. El universo estuvo constituido por menores de un año diagnosticados de fisura labial unilateral incompleta, que asistieron a la consulta de Cirugía Maxilofacial de dicho hospital, en el período en que se realizó la investigación y en los que para su tratamiento se empleó la técnica de Rotación y Avance de Millard modificada por Mulliken. Se trabajó con la totalidad del universo. Se utilizó estadística descriptiva. Los pacientes fueron intervenidos entre los 5 y 9 meses, predominó el color de piel blanca (75 %) y el sexo masculino (75 %). La altura labial y el ancho heminasal aumentaron más en el lado sano que en el fisurado. Al año de la intervención en el lado fisurado creció más el ancho transversal que en el sano en dos de los casos y también existió una diferencia mayor de 1 mm en la altura del bermellón. Se logró alcanzar simetría nasolabial en espejo del lado sano en todas las variables, excepto en la altura del bermellón en dos casos.

ABSTRACT The biological, social, economic and psychological impact caused by cleft lip leads to the application of new modifications in the surgical correction of its treatment techniques. The objective of this research was to evaluate the results of the Millard's rotation-advancement surgical technique modified by Mulliken in the correction of incomplete unilateral cleft lip in Matanzas. A longitudinal, prospective study was conducted at "Eliseo Noel Caamaño" Pediatric Teaching Hospital, in Matanzas between 2016 and 2018. The universe was made up of children under one year of age diagnosed with incomplete unilateral cleft lip, who come to the Maxillofacial Surgery consultation of this hospital, in the period in which the investigation was carried out and in whom, Millard 's rotation-advancement surgical technique modified by Mulliken was used for their treatment. We worked with the entire universe. Descriptive statistics was used. The patients underwent surgery between 5 and 9 months, white skin color (75%) and male gender (75%) predominated. Labial height and heminasal width increased more on the healthy side than on the cleft side. One year after the intervention, the transverse width grew more on the cleft side than on the healthy side in two of the cases, and there was also a difference greater than 1 mm in the vermilion height. Mirror-image nasolabial symmetry was achieved on the healthy side in all variables, except for vermilion height in two cases.

Análise Descritiva da Ocorrência de Fenda Orofacial ­ Série Histórica de 2007 a 2016 no Sul do Brasil / Descriptive Analysis of the Occurrence of Orofacial Cleft - Historical Series from 2007 to 2016 in Southern Brazil

Introdução: Entre a quarta e décima segunda semana de gestação, ocorre o desenvolvimento da cavidade bucal e da face. Caso aconteça alguma desordem nesse período, acarretará a formação de fissuras labiopalatinas, uma das mais frequentes anomalias congênitas orofaciais. Este estudo teve como objetivo analisar a tendência temporal de fissura labiopalatina no sul do Brasil, durante o período de 2007-2016, e caracterizar o perfil epidemiológico do recém-nascido e da mãe dos portadores dessa malformação. Métodos: Estudo descritivo de tipo ecológico, tendo como fonte de dados o Sistema de Informações de Nascidos Vivos (SINASC). Foram pesquisados 3203 nascimentos com fenda orofacial no sul do Brasil durante o período estudado. Resultados: Observou-se uma homogeneidade em relação à incidência dessa malformação nos três estados estudados ­ 8,39 casos a cada 10.000 nascimentos, com leve predomínio em Santa Catarina. Quanto ao perfil epidemiológico dos recém-nascidos, notou-se uma incidência 37% maior no sexo masculino do que no sexo feminino. Além disso, a incidência dessa malformação ocorreu mais em indivíduos com baixo peso ao nascer e em prematuros. Em relação ao perfil epidemiológico das mães dos recém-nascidos com fissura labiopalatina, a idade materna apresentou um pico bimodal, com incidência 21% maior em mulheres com menos de 14 anos do que com mais de 35 anos. Conclusão: Este estudo demonstrou fatores de risco para fissura labiopalatina como: sexo masculino, baixo peso ao nascer, parto cesário, pré-natal incompleto, prematuridade, extremos de idade materna e baixo grau de instrução materna.

Introduction: Between the fourth and twelfth week of gestation, the development of the oral cavity and face occurs. If any disorder occurs during this period, it will lead to cleft lip and palate formation, one of the most frequent congenital orofacial anomalies. This study aimed to analyze the temporal trend of cleft lip and palate in southern Brazil from 2007 to 2016 and to characterize the epidemiological profile of the newborn and the mother of carriers of this malformation. Methods: A descriptive study of ecological type using the Live Births Information System (SINASC) as a data source. A total of 3203 births with orofacial cleft were surveyed in southern Brazil during the period studied. Results: Homogeneity was observed regarding the incidence of this malformation in the three states studied - 8.39 cases per 10,000 births, with a slight predominance in Santa Catarina. Concerning the epidemiological profile of the newborns, we observed a 37% higher incidence in males than in females, and the incidence of this malformation occurred more in individuals with low birth weight and premature babies. Regarding the epidemiological profile of mothers of newborns with cleft lip and palate, maternal age showed a bimodal peak, with a 21% higher incidence in women aged under 14 than in those over 35. Conclusion: This study demonstrated risk factors for cleft lip and palate, such as male gender, low birth weight, cesarean delivery, incomplete prenatal care, prematurity, extremes of maternal age, and low maternal education.

Rinoplastia secundaria en pacientes con fisura labiopalatina / Secondary Rhinoplasty in Patients with Clef lip and Palate

Introducción: La deformidad nasal asociada con el labio leporino ha sido vista como uno de los problemas reconstructivos más desafiantes en la rinoplastia, hasta el momento no se ha acordado ningún método único para cuantificar el éxito del tratamiento. En el año 2006 se aprobó oficialmente el protocolo para el tratamiento de las deformidades nasales complejas del Hospital Clínico Quirúrgico "Hermanos Ameijeiras", que están incluidas, las deformidades nasales congénitas secundarias en el adulto, no se recogen antecedentes de resultados en este grupo de pacientes basados en mediciones objetivas. Objetivo: Evaluar los resultados estéticos de la rinoplastia secundaria en pacientes con fisura labiopalatina. Métodos: Se realizó un estudio analítico a través de mediciones nasales angulares y de áreas, comparadas en fotografías estandarizadas entre el pre y posoperatorio y se determinó el índice de asimetría según la fórmula de Nakamura. Un panel de expertos usó la escala de Asher McDade y realizó la evaluación subjetiva de las imágenes, que permitió calcular el índice estético antes y después del tratamiento. Resultados: Se obtuvo una reducción marcada de todos los índices de asimetría nasal entre el pre y posoperatorio, así como una mejoría del índice estético posoperatorio pasándose de una apariencia pobre a muy buena apariencia. Conclusiones: Con la aplicación del protocolo para el tratamiento de las deformidades nasales del paciente fisurado adulto del Hospital Clínico Quirúrgico "Hermanos Ameijeiras" se obtienen buenos resultados estéticos(AU)

Introduction: Nasal deformity associated with cleft lip has been seen as one of the most challenging reconstructive problems in rhinoplasty; up to date, no single method has been agreed upon to quantify management success. In 2006, the protocol for managing complex nasal deformities was officially approved by Hermanos Ameijeiras Clinical Surgical Hospital, which includes secondary congenital nasal deformities in adults; however, there is no history of outcomes in this group of patients based on objective measurements. Objective: To assess the aesthetic outcomes of secondary rhinoplasty in patients with cleft lip and palate. Methods: An analytical study was carried out through linear and angular nasal measurements, compared in standardized photographs between the pre- and postoperative period; while the asymmetry index was determined according to Nakamura's formula. A panel of experts used the Asher McDade scale and performed the subjective assessment of the images, which allowed calculation of the esthetic index before and after the procedure. Results: A marked reduction was obtained in all nasal asymmetry indexes between the pre- and postoperative periods; as well as an improvement in the postoperative aesthetic index, from a poor appearance to a very good appearance. Conclusions: With the application of the protocol for managing nasal deformities of the adult cleft patient of Hermanos Ameijeiras Clinical Surgical Hospital, good esthetic outcomes are obtained(AU)

Evaluation of palatal volume in children with cleft lip and palate: a comparison of two surgical protocols / Avaliação volumétrica do palato em crianças com fissura labiopalatina: comparação de dois protocolos cirúrgicos

Objective: Compare the palatal volume in children with unilateral cleft lip and palate before and after two surgical protocols. Material and Methods: Retrospective data collection was performed in a specialized hospital. The sample comprised 120 digitized dental models divided into, Group 1 (G1) ­ participants submitted to cheiloplasty at 3 months (Millard technique) and one-step palatoplasty at 12 months (von Langenbeck technique); Group 2 (G2) ­ participants submitted to cheiloplasty (Millard technique) and hard palate closure (Hans Pichler technique) at 3 months and soft palate closure at 12 months (Sommerlad technique). The dental models were evaluated at Time 1 (T1): before primary plastic surgeries, Time 2 (T2): 1st post-surgical phase, and Time 3 (T3): 2nd post-surgical phase. The volume was measured through stereophotogrammetry system software. Parametric and non-parametric statistical tests were applied (α=5%). Results: The intragroup analysis revealed that G1 had a statistically significant increase in volume at T2 followed by a reduction at T3 (p=0.003); G2 showed a statistically significant increase of dental arch volume between T1 and T2 (p=0.001). There was no statistically significant difference in the intergroup and gender analyses (p>0.05). Conclusion: The surgical protocol influenced the palatal volume of children with unilateral cleft lip and palate. This study suggested that two-step palatoplasty protocol has a tendency to be more appropriate.(AU)

Objetivo: Comparar o volume palatino em crianças com fissura unilateral de lábio e palato antes e após dois protocolos cirúrgicos. Material e Métodos: A coleta de dados retrospectiva foi efetuada em um hospital especializado. A amostra foi composta por 120 modelos dentários digitalizados divididos em, Grupo 1 (G1) ­ participantes submetidos a queiloplastia aos 3 meses de vida (técnida de Millard) e a palatoplastia em única etapa aos 12 meses (técnica de von Langenbeck); Grupo 2 (G2) ­ participantes submetidos a queiloplastia (técnica de Millard) e fechamento do palato duro (técnica de Hans Pichler) aos 3 meses de vida e fechamento do palate mole aos 12 meses (técnica de Sommerlad). Os modelos dentários foram avaliados em Tempo 1 (T1): antes das cirurgias plásticas primárias, Tempo 2 (T2):1ª fase pós-cirúrgica e Tempo 3 (T3): 2ª fase pós-cirúrgico. O volume foi mensurado por meio do software do sistema de estereofotogrametria. Testes estatísticos paramétricos e não-paramétricos foram utilizados (α=5%). Resultados: As análises intragrupos indicaram que G1 apresentou aumento estatisticamente significante em T2 seguido de redução em T3 (p=0.003). G2 apresentou crescimento estatisticamente significativo do volume palatino entre T1 e T2 (p=0.001). Não houve diferença estatisticamente significante nas análises intergrupos e entre gêneros (p>0.05). Conclusão: O protocolo cirúrgico influenciou o volume palatino das crianças com fissura unilateral de lábio e palato. Este estudo sugeriu que o protocolo da palatoplastia em duas etapas possui uma tendência de ser mais apropriado.(AU)