Digital nasoalveolar molding through presurgical orthopedics in newborns/infants with cleft lip and palate: A comprehensive review and case study.

AIMS: This study aims to provide a comprehensive review and case study about the advantages and disadvantages of the application of digital technologies in presurgical orthopedics in newborns/infants with cleft lip and palate (CLCP). Positive changes in the nasal anatomy, maxillary arch, and cleft width could be achieved. METHODS: Three representative cases of newborns/infants with CLCP were managed using the presurgical newborn/infant orthopedics (PSIO) approach. The patients were diagnosed and treated. Detailed descriptions of the impression procedures and PSIO appliance construction and placement were provided for each case. RESULTS: Case 1 utilized traditional impression techniques, Case 2 employed a semi-digitalized approach with intraoral digital scanning, and Case 3 utilized a completely digitalized method for appliance construction. Positive changes in maxillary arch dimensions and cleft width reduction were observed in all cases. CONCLUSIONS: The management of CLCP in newborns and infants poses a complex challenge with profound implications. The PSIO approach not only facilitates reconstructive surgery but also enhances overall quality of life. Digital tools, like specialized optical scanners and 3D printing, revolutionize the PSIO process, making it more efficient and patient-friendly. Clinical benefits include improved facial morphology, esthetics, feeding, speech, and optimized future surgical results. Despite ongoing efficacy debates, global adoption as the initial surgical approach underscores its value. The integration of digital technologies offers new hope for patients and families, promising a brighter future for those affected by this congenital condition.

Association of methylenetetrahydrofolate reductase gene variant C677T and folate levels in non-syndromic cleft lip/palate among Sindhi, Pakistani population.

The object ives of this study were to determine the association of methylenetetrahydrofolate reduc tase (MTHFR) gene variant C67 7 T with non -syndromic cl eft lip/palate (NSCLP) in Pakistani population and compare the m aternal serum foli c acid levels in NSCLP-affected and healthy group. A c om parative cross sec ti onal study was conducted between 2017 and 2019 at Liaquat U niversity of Medi cal and Health Science s, Jamshoro. Sixty motherinfant dy ads were recruited (n=120), inc luding NSCLP-affected and healthy infants alo ng with t heir mother s. The MTHFR C677T vari ant exhibited si gnificant association with NSCLP in dominant and over-domi nant models. No differences in maternal serum folic acid levels were obse rved between both th e groups; however, the folic acid intake during pre-conception period was associated w ith decreased risk for NSC LP. Our stu dy suggested that MTHFR 677 CT genotype was related with decreased risk for NSCLP in Sindhi, Pakistani, population. Pre -conception folic acid may decrease the ri sk for ora l clefts.

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.

Dental Anomalies and Muscle Segment Homeobox1 Gene Polymorphism in Nonsyndromic Cleft Lip with or without Palate Children

@#This study aims to determine the prevalence of dental anomalies and MSX1 gene 799G>T polymorphism and its association with non-syndromic cleft lip with or without palate (NSCLP) attending Hospital Universiti Sains Malaysia. Clinical and radiological assessments on 37 NSCL±P patients and 80 non-cleft children were done to detect dental anomalies. The buccal cells were collected and polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) was used to identify polymorphism. NSCL±P was higher among males (54%) and mostly unilateral cleft lip and palate (51.3%). The prevalence of dental anomalies in morphology in NSCL±P was 18.9% (95% CI: 5.7%, 32.2%) and non-cleft was 6.3% (95% CI: 0.8%, 11.7%). Hypodontia in NSCLP was 75% (95% CI: 61.2%, 90.2%) and non-cleft was 7.5% (95% CI: 1.6%, 13.4%). There was a significant association between NSCLP and anomalies in morphology (P= 0.04; OR=3.5)) and number (P< 0.01; OR= 40). There was an absence of rare 799G>T polymorphism in all NSCL±P and non-cleft children indicating that all samples contain common 799G polymorphism. In conclusion, the prevalence of dental anomalies in morphology and number was significantly higher in NSCL±P compared to non-cleft children. However, it was not significantly associated with MSX1 799G>T polymorphism.

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.

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.

A Baby Born with Ectopia Cordis, Omphalocele, Cleft Lips and Palate: A Case Report.

Ectopia cordis is a rare congenital defect with the prevalence of 5 to 8 per million live births. Here we report a rare case of preterm female live birth with ectopia cordis associated with omphalocele, cleft lip, and palate. In this case, 14+ weeks ultrasound did not show any fetal abnormalities and parents were unaware of the condition until 35+ weeks when ultrasound detected the anomaly a few days before delivery. After delivery, they didn't give consent for further intervention which led to neonatal mortality 3 hours after birth. If the condition was diagnosed in time, an earlier intervention could have been done.

The role of surgeon's learning on the outcomes of alveolar bone graft for cleft repair.

BACKGROUND AND PURPOSE: Teaching cleft repair in the operating room may not sufficiently prepare craniofacial residents to perform the procedure self-reliantly. This study aimed to assess the influence of surgeons' experience level on the operative time and graft survival ratio after alveolar bone grafts for cleft repair. SUBJECTS AND METHODS: This was a retrospective review of 100 consecutive alveolar bone graft patients treated for unilateral alveolar cleft by one craniofacial surgeon between 2012 and 2019. An alveolar bone graft from the iliac bone was performed using the lateral sliding flap. Sex, age, cleft width, cleft defect volume, and operative time were recorded, and the 1-year postoperative graft survival ratio was calculated. Multiple regression analysis was performed to identify factors that affect the operative time and survival ratio. RESULTS: Factors affecting the operative time were the experience level of the surgeon and the presence of a cleft palate. Factors affecting the graft survival ratio were the cleft defect volume and experience level of the surgeon. In cleft lip and alveolus, the survival ratio was constant, and the operative time stabilised after approximately 20 cases. In cleft lip and palate, more experience was required to master the technique, and the operative time and survival ratio were stabilised only after approximately 40 cases. CONCLUSION: Surgeons' experience level influenced the outcomes of alveolar bone grafting. Although surgical training is currently undergoing a paradigm shift from on-the-job training to simulation-based training, clinical experience is critical to be self-sufficient.

Association of social and psychological aspects of quality of life and educational level of patients with different orofacial cleft.

OBJECTIVE: To determine the social and psychological domains of quality of life of children with orofacial clefts in the context of different types of clefts and educational levels. METHODS: The cross-sectional study was conducted at Clapp Hospital and Mayo Hospital, Lahore, Pakistan from September 1, 2020, to January 31, 2021, and comprised subjects of either gender aged 6-18 years having orofacial clefts. Data was collected using the CLEFT-Questionnaire along with a basic demographic sheet. Data was analysed using SPSS 23. RESULTS: Of the 80 subjects, 40(50%) each were males and females. The overall mean age was 12.41±3.39 years. A significant association of types of orofacial clefts with social function (p<0.05) and psychological function (p<0.05) was observed. The highest mean score of 27.89±3.41 and 26.11±1.76 was noted for unilateral left side cleft lip, and primary palate, respectively. No significant association of level of education with social function (p>0.05) and psychological function (p>0.05) was noted. CONCLUSIONS: Different types of orofacial clefts affected the psychological and social aspects of quality of life of patients differently, but the difference was not significantly correlated with the education level.

Response to Pulp Vitality Tests in Teeth Submitted to Orthodontic Movement, Adjacent to the Cleft Area.

OBJECTIVE: To evaluate the pulp vitality in teeth adjacent to the cleft area submitted to orthodontic movement into the alveolar graft area in individuals with complete unilateral cleft lip and palate (CUCLP). DESIGN: Cold sensitivity, vertical, and horizontal percussion tests were conducted on the teeth adjacent to the cleft and the contralateral teeth. SETTING: Endodontics Sector in the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo (HRAC/USP). PATIENTS: One hundred patients with CUCLP and hypodontia of the upper lateral incisor in orthodontic movement and after successful alveolar bone graft in the cleft area. MAIN OUTCOME MEASURES: The cleft study group (SG) was composed of 200 teeth, adjacent to the cleft area. The control group (CG) consisted of 200 contralateral teeth. Statistical analysis was performed using the chi-square test for comparisons between groups (P < .05). RESULTS: In the SG, 82.0% of teeth presented positive response to the cold sensitivity testing, 13.5% had negative response, and 4.5% had marked response, with statistically significant difference in relation to the CG. The vertical and horizontal percussion tests on teeth in the SG revealed the same results, in which 95.0% presented negative response and 5.0% responded positively, without significant difference compared to teeth in the CG, for both tests. CONCLUSIONS: Teeth adjacent to the cleft area presented changes in the physiological conditions of the pulp, which were observed by reduction of positive response to the cold sensitivity testing or presence of pulp hypersensitivity in cases of marked response.

Post-residency fellowship training in Maxillofacial Prosthetics is needed in Pakistan.

OBJECTIVE: To determine the spectrum of maxillofacial prosthetics services in Pakistan, to explore the need of a relevant fellowship training programme in the country, and to determine whether they contribute to tumour board of head and neck oncology. METHODS: The cross-sectional online survey was conducted from March to June 2020 and comprised all prosthodontists registered on the portal of the Pakistan Prosthodontics Association. Using Google Forms, the survey questionnaire was sent via email. The survey form was self-generated. Data was analysed using SPSS 23. RESULTS: Of the 84 prosthodontists approached, 44(52.4%) responded. The overall median age was 39 years (range: 30-60 years), and 34(77.3%) subjects were males. Overall, 19(43.2%) subjects were from Punjab, 14(31.8%) Sindh, 6(13.6%) Khyber Pakhtunkhwa, and 5(11.4%) Islamabad. None of the participants had received any formal accredited training in maxillofacial prosthetics. Although 37(84.1%) respondents reported providing maxillofacial prosthetics services to their patients as they had learnt it during their prosthodontics residency. Only 3(6.8%) respondents were contributing to head and neck tumour board. An overwhelming majority 42(95.5%) stressed the need of structured training programmes in maxillofacial prosthetics. CONCLUSIONS: There is no accredited maxillofacial prosthetics training centre and an obvious lack of prosthodontists with formal training in the professional area. The participation of prosthodontists in the head and neck tumour board was negligible.

Occipital Encephalocele with Multiple Birth Defects: A Case Report.

A full-term female baby presented at 24 hours of life at the emergency department with occipital encephalocele, bilateral cleft lip, and cleft palate. She was born to a second gravida mother with no consanguinity between the parents. On examination, encephalocele was 10centimeters x 7centimeters in size with bilateral cleft lip and palate. It presents the opportunity for healthcare professionals to learn about a group of congenital neurological disorders in the content of a rare case presentation and highlights the importance of ultrasonography in the antenatal period for the detection of neural tube defects in the early stage for proper counselling and management. A compulsory prenatal diagnosis of the suspected family should be done by the intervention of the public sector of any country so that we can prevent and avoid abnormal birth.

Abordagem fonoaudiológica nas fissuras orofaciais não sindrômicas: revisão de literatura / Speech pathology treatment on nonsyndromic orofacial clefts: literature review

A fi ssura labiopalatina é uma malformação comum que ocorre em virtude da falta de fusão dos processos embrionários que formam a face e o palato. A avaliação e o tratamento voltados à fi ssura labiopalatina envolvem uma equipe multidisciplinar que contempla, entre outras especialidades, cirurgião-plástico, fonoaudiólogo, ortodontista, cirurgião-bucomaxilofacial, cirurgião-dentista, odontopediatra, otorrinolaringologista e psicólogo. Desse modo, por meio da assistência com profi ssionais especializados, é possível alcançar resultados estéticos e funcionais satisfatórios, proporcionando interações sociais mais efetivas e consequentemente melhor qualidade de vida ao fi ssurado. O objetivo deste trabalho é apresentar alguns dos princípios da ação do fonoaudiólogo nas fi ssuras orofaciais não sindrômicas, destacando-se a avaliação e terapia voltadas à função alimentar, mecanismo velofaríngeo e aspectos articulatórios da fala. Para tanto, procedeu-se a uma revisão narrativa da literatura. Como critério de inclusão dos trabalhos, consideraram-se publicações em coletâneas de repercussão na área fonoaudiológica e manuscritos que contemplassem os seguintes temas: aleitamento, orientações pré e pós-cirúrgicas, articulações compensatórias, função velofaríngea e terapia fonoaudiológica.

Cleft lip and palate is a common defect that occurs due to the lack of fusion of embryonic processes that form the face and the palate. Evaluation and treatment for cleft lip and palate involve an interdisciplinary team that includes, among other specialties, plastic surgeon, speech-language therapist, otorhinolaryngology, audiologist, orthodontist, maxillofacial surgeon and psychologist. Thus, through assistance with specialized professionals, it is possible to achieve satisfactory results, providing more effective social interactions and consequently better quality of life to subject with cleft lip and palate. The aim of this paper is to present some of the speech-language therapist's action principles on typical orofacial clefts, emphasizing the evaluation and therapy focused on food function, velopharyngeal mechanism and articulatory aspects of speech. Therefore, we proceeded to a literature review. For the inclusion of the papers, the following topics were considered: breastfeeding, pre and post-surgical guidelines, compensatory articulation, velopharyngeal function and speech-language therapy

Postoperative long-term results for the comparison of the symmetry of the upper lip during lip closure according to Millard and Pfeifer.

BACKGROUND: Clefts in newborns are associated with severe morphological and functional impairment. Especially the lip is of importance as if the treatment result is unsatisfactory, it can lead to psychological changes in the patient. Different operative procedures have been developed over the last decades. The aim of the presented study was the comparison of the surgical techniques according to Millard and Pfeifer regarding the temporal development of the postoperative symmetry of the lip height and mouth width. METHODS: Digitized photographs of patients from the department of oral and maxillofacial surgery at the University of Göttingen were evaluated from 1979 to 1996. With a video analysis program, the lip height and mouth width were analyzed regarding the symmetry. We demonstrated the symmetry values over a period of 8 years in order to show the influence of growth on postoperative results. RESULTS: The development of the vertical symmetry of the Philtrum and the lip vermillion on the cleft side in comparison to the healthy side behaves differently depending on Pfeifer and Millard. The lip height of the cleft lip was shorter in both techniques than on the healthy side, but Pfeifer's difference was significantly more pronounced. The lip vermillion height on the cleft side was slightly shorter in the Millard group and markedly larger in the Pfeifer group. Both techniques can achieve good symmetry results for the vertical dimension of the lip. According to Pfeifer, the development of the horizontal dimension on the cleft side is bigger within the first 4 years than on the healthy side; according to the Millard technique, the horizontal development is smaller. These differences are greater within the first 6 years and approach between the 6th and 8th year. CONCLUSIONS: The Millard technique demonstrates better results concerning the philtrum and vermillion symmetry during growth within the first 6 years. Over the whole study period, growth corrects the philtrum and vermillion symmetry within the Pfeifer group.

Aesthetic satisfaction in lip and palate clefts: a comparative study between secondary and tertiary bone grafting.

Lip and palate cleft represent one of the most frequently occurring congenital deformity, which includes dental anomalies, such as variation in tooth number and position. In case of hypodontia implant-prosthetic rehabilitation offers significant advantages in terms of function, aesthetics and quality of life and bone graft is usually needed. Secondary bone grafting, generally performed in the mixed dentition phase (years 8-11) seems to be the most successful method to allow for rehabilitation. It's often necessary to perform a tertiary bone grafting in adult age in order to achieve better bone quantity and quality before implant placement. Aim of this retrospective study was to evaluate the aesthetic perception that patients had of themselves comparing dental implants placed in tertiary grafted alveolar cleft sites with a previous secondary grafting to only secondary grafting. Between 2009 and 2012, fourteen alveolar cleft were treated with implant rehabilitation and eleven of them received tertiary bone grafting six months prior to implant placement. All patients were questioned to give a score from 1 to 10 their aesthetic satisfaction of their smile before and after implant rehabilitation and during pre-surgery provisional rehabilitation. At the end of their prosthesis rehabilitation patients who received tertiary bone grafting resulted more satisfied than those who had secondary bone grafting only (9.5 vs 8).

Rare Variant of Ankyloblepharon-ectodermal Defect-cleft Lip/Cleft Palate Syndrome: Curly Hair-ankyloblepharon-nail Disease Syndrome.

Ankyloblepharon-ectodermal defect-cleft lip/cleft palate (AEC) syndrome is one of the variants of ectodermal dysplasia. It is an autosomal dominant disorder comprising of ankyloblepharon, ectodermal dysplasia, and cleft palate or cleft lip. In 1976, it wasfirst described by Hay and Wells, therefore also known as Hay-Wells syndrome. The characteristic feature of this syndrome is "ankyloblepharon filiforme adnatum", which refers to the partial thickness fusion of the eyelid margins. The "curly hair-ankyloblepharon-nail disease (CHAND) syndrome" is a clinical variant of AEC syndrome. We report a rare case of a 7-year-old girl child who presented with history of abnormal dentition, 20 nail dystrophy, and light-colored, sparse curly hairs since birth. Parents gave history that at the time of birth, her both eyelids were fused partially, which was surgically corrected by an ophthalmologist at 1 month of age. There was no history of hypohidrosis or anhidrosis, heat intolerance, cleft lip or cleft palate. Microscopy of the hair shaft found "bubbly hair" morphology. This case is unique as it is a rare presentation, and awareness should be there for this constellation of findings so that the systemic associations can be investigated. "Bubble hair" morphology on microscopy is a unique feature in this rare autosomal recessive condition.

Postoperative long-term results for the comparison of the symmetry of the upper lip during lip closure according to Millard and Pfeifer / 대한악안면성형재건외과학회지

BACKGROUND: Clefts in newborns are associated with severe morphological and functional impairment. Especially the lip is of importance as if the treatment result is unsatisfactory, it can lead to psychological changes in the patient. Different operative procedures have been developed over the last decades. The aim of the presented study was the comparison of the surgical techniques according to Millard and Pfeifer regarding the temporal development of the postoperative symmetry of the lip height and mouth width. METHODS: Digitized photographs of patients from the department of oral and maxillofacial surgery at the University of Göttingen were evaluated from 1979 to 1996. With a video analysis program, the lip height and mouth width were analyzed regarding the symmetry. We demonstrated the symmetry values over a period of 8 years in order to show the influence of growth on postoperative results. RESULTS: The development of the vertical symmetry of the Philtrum and the lip vermillion on the cleft side in comparison to the healthy side behaves differently depending on Pfeifer and Millard. The lip height of the cleft lip was shorter in both techniques than on the healthy side, but Pfeifer's difference was significantly more pronounced. The lip vermillion height on the cleft side was slightly shorter in the Millard group and markedly larger in the Pfeifer group. Both techniques can achieve good symmetry results for the vertical dimension of the lip. According to Pfeifer, the development of the horizontal dimension on the cleft side is bigger within the first 4 years than on the healthy side; according to the Millard technique, the horizontal development is smaller. These differences are greater within the first 6 years and approach between the 6th and 8th year. CONCLUSIONS: The Millard technique demonstrates better results concerning the philtrum and vermillion symmetry during growth within the first 6 years. Over the whole study period, growth corrects the philtrum and vermillion symmetry within the Pfeifer group.

Acción de tutela y barreras de acceso a servicios de salud en labio y/o paladar hendido / Tutela and acces barriers to helth services to cleft lip and palate / Ação de tutela 1 e barreiras de acesso aos serviços de saude em pacientes com fissuras labiopalatinas

La investigación busca identificar barreras de acceso a servicios de salud para la atención integral de pacientes con labio y/o \r\npaladar hendido (LPH) a partir del análisis de acciones de tutela relacionadas con esta condición en un periodo de veinte años: \r\n1994-2014. Se identificaron tutelas interpuestas por familiares de niños con LPH; se construyó una matriz para organizar los datos \r\nmás relevantes (motivos de tutela, fallo y consideraciones para este); posteriormente, se hizo un análisis para identificar las barreras \r\ny la manera como la justicia resuelve la solicitud. En total, se analizaron once fallos de tutela. El principal motivo para interponerlas \r\nfue la negación por parte de la entidad promotora de salud de tratamientos para LPH por no encontrarse incluidos en el plan de \r\natención en salud (catalogados como estéticos) o por falta de infraestructura para su atención. Cinco de ellas fueron negadas por \r\nrazones administrativas o procedimentales, por dificultades en la afiliación a una entidad de salud o porque cesaron los efectos \r\nque motivaron la tutela. En las tutelas aceptadas, se obliga a realizar la atención integral, considerando la garantía del derecho a la \r\nsalud en conexidad con los derechos a la vida, la dignidad y los derechos de los niños. Conclusión: la principal barrera de atención para pacientes con LPH se relaciona con el hecho de que algunos tratamientos no están incluidos en el plan de atención en salud \r\nde Colombia; por tanto la acción de tutela, a la luz del derecho a la salud, permite aclarar imprecisiones del Sistema General de \r\nSeguridad Social y superar las barreras en la atención de individuos con labio y/o paladar hendido.

Objective: to identify access barriers to health services to \r\nachieve comprehensive care of patients with Cleft Lip and/or \r\nPalate (CLP), based on a "tutela," or writ of fundamental rights, \r\nanalysis related with this condition in a 20-year period, from \r\n1994 to 2014. Materials and methods: tutelas from parents of \r\nchildren with CLP were identified; a matrix to organize the \r\nrelevant data (reasons for the tutela, verdict and related consid\r\n-\r\nerations) was developed and an analysis carried out to identify \r\nthe barriers and how the issues were legally resolved. Results: \r\nthere were 11 tutelas reviewed and the main reasons for their \r\nfilings were: negation of treatment for conditions related to \r\nCLP, as they were not included in the health plan that regarded \r\nthe treatment as aesthetic intervention, or due to lack of infra\r\n-\r\nstructure for their attention. Five were denied due to lack of \r\nawareness of the attending agency, difficulties in affiliation to \r\na health provider, or due to cessation of the effects that led \r\nto the tutela. In those, which were accepted, comprehensive \r\nhealth was mandated, taking into consideration the right of \r\naccess to health in relation to the rights of life, dignity and chil\r\n-\r\ndren's rights. Conclusion: the main barrier to care for patients \r\nwith CLP is related to the fact that some treatments are not \r\nincluded in the health care plan of Colombia; therefore the \r\ntutela, in light of the right to health, can clarify ambiguites of \r\nthe General System of Social Security and overcome barriers in \r\nthe care of individuals with cleft lip and/or palate.

Objetivo: identificar barreiras de acesso aos serviços de saúde \r\npara atenção integral de pacientes com fissuras labiopalatinas \r\n(FLPs), partindo da análise das ações do procedimento cons\r\n-\r\ntitucional relacionadas com essa condição, em um período \r\nde 20 anos: 1994-2014. Materiais e métodos: identificaram-se \r\nprocedimentos constitucionais interpostos pela família das \r\ncrianças com FLPs; foi criada uma matriz para organizar os \r\ndados mais relevantes (motivos do procedimento constitu\r\n-\r\ncional, conclusão e considerações para esta) logo, foi feita uma \r\nanálise para identificar as barreiras e o modo como a justiça \r\nas resolve. Resultados: foram analisados 11 procedimentos \r\nconstitucionais. As principais razões para interpô-los: negação \r\ndos tratamentos para FLPs, por não se encontrarem inclusas \r\nno plano de saúde (estéticos) ou pela ausência de infraestru\r\n-\r\ntura para seu atendimento. Cinco deles foram negados: pelo \r\ndesconhecimento perante à entidade encarregada da atenção, \r\ndificuldades na afiliação a um plano de saúde ou porque \r\ncessaram os efeitos que os motivaram. Nos aceitos, obriga-\r\nse a realizar a atenção integral, considerando-se a garantia \r\ndo direito em conexão com os direitos à vida, dignidade e \r\ndireitos das crianças. Conclusão: A principal barreira para cuidar \r\nde pacientes com FLP está relacionado com o fato de que \r\nalguns tratamentos não estão incluídos no plano de saúde \r\nda Colômbia; portanto, a tutela, à luz do direito à saúde, pode \r\nesclarecer imprecisões do Regime Geral da Segurança Social \r\ne superar as barreiras no cuidado aos indivíduos com fissuras \r\nlabiopalatinas.

Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip-Cleft Palate.

Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure.

Lower lip cleft, bifid tongue and fibrolipoma: a case report of rare congenital anomaly.

A non-syndromic bifid tongue with fibrolipoma, ankyloglossia, and clefts of the lower lip and alveolus is rare. We describe a case in which a bifid anterior tongue was separated by a fibrolipomatous mass. We excised the mass and repaired the lip and the tongue with satisfactory results.