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Introdução: Muitos pacientes portadores de sequência de Pierre Robin (micrognatia, glossoptose e obstrução de via aérea) apresentam o músculo genioglosso alterado, encurtado e retrátil, que impede a protração lingual, mantendo a parte anterior da língua verticalizada e seu volume deslocado em direção posterior. Isso pode corroborar para obstrução supraglótica, dificuldade alimentar e inversão das forças de estímulo do crescimento do corpo mandibular. Métodos: Estudo retrospectivo de pacientes com Pierre Robin tratados entre 2012 e 2017 pela equipe, com descrição da "ortoglossopelveplastia", que propõe uma modificação na glossopexia, soltando o genioglosso anômalo da sua inserção, liberando a língua para elevar seu terço anterior e avançar o volume de sua base, sendo auxiliada por ponto de tração da base lingual à sínfise mandibular. Apresentamos um algoritmo de tratamento proposto que prioriza a necessidade desta cirurgia, associada ou não à distração mandibular, de acordo com a gravidade da dificuldade respiratória e/ou alimentar. Resultados: São apresentados 12 casos de obstrução da orofaringe atendidos de 2012 a 2017, discutem-se suas prioridades, a ortoglossopelveplastia e se aplica o algoritmo proposto. Conclusão: A reorganização anatômica da musculatura em uma posição anteriorizada correta proporciona protração e funcionalidade à língua, com desobstrução da via aérea na orofaringe, melhora da função alimentar e do desenvolvimento mandibular, com baixa morbidade cirúrgica e poucas complicações.

Introduction: Several patients with the Pierre Robin sequence (micrognathia, glossoptosis, and airway obstruction) have an altered, shortened, and retractable genioglossus muscle that prevents protraction of the tongue and keeps the anterior part of the tongue vertical and its volume posteriorly displaced. This can contribute to supraglottic obstruction, feeding difficulty, and inversion of the growth stimulation forces of the mandibular body. Methods: A retrospective study of patients with the Pierre Robin sequence treated between 2012 and 2017 with "orthoglossopelveplasty," which includes modification of glossopexy, releasing the anomalous genioglossus of its insertion and releasing the tongue to raise its anterior third and advance the volume of its base using a traction suture of the tongue base to the mandible symphysis. We present a treatment algorithm that prioritizes the need for surgery associated, or not, with mandibular distraction in accordance with respiratory and/or feeding difficulty severity. Results: Twelve cases of oropharyngeal obstruction treated from 2012 to 2017 are presented, their priorities and orthoglossopleoplasty are discussed, and the proposed algorithm is applied. Conclusion: Anatomical reorganization of the musculature in a correct anterior position provides protraction and functionality to the tongue, clears the airway in the oropharynx, and improves the feeding function and mandibular development, with low surgical morbidity rates and few complications.

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Abstract Introduction: Children may require tracheostomy due to many different health conditions. Over the last 40 years, indications of tracheostomy have endorsed substantial modifications. Objective: To evaluate pediatric patients warranted tracheostomy at our Hospital, in regard to their indications, associated comorbidities, complications and decannulation rates. Methods: Retrospective study concerning patients under 18 years of age undergoing tracheostomy in a tertiary health care center, from January 2006 to November 2015. Results: 123 children required a tracheostomy after ENT evaluation during the study period. A proportion of 63% was male, and 56% was under one year of age. Glossoptosis was the most common indication (30%), followed by subglottic stenosis (16%) and pharyngomalacia (11%). The mortality rate was 31%. By the end of this review, 35 children (28.4%) had been decannulated, and the fewer the number of comorbidities, the greater the decannulation rate (0.77 ± 0.84 vs. 1.7 ± 1.00 comorbidities; p < 0.001). Conclusion: Tracheostomy in children is a relatively frequent procedure at our hospital. The most common indications are glossoptosis and subglottic stenosis. A high mortality rate was found, potentially substantiated by the high number of critical care patients with chronic neurological conditions in this cohort. Our decannulation rate is slightly below other series, probably because of the greater amount of patients with comorbidities.

Resumo Introdução: As crianças podem necessitar de traqueostomia devido a diferentes problemas de saúde. Ao longo dos últimos 40 anos, as indicações de traqueostomia passaram por mudanças substanciais. Objetivo: Avaliar pacientes pediátricos com traqueostomia no nosso hospital, no que diz respeito às suas indicações, comorbidades associadas, complicações e taxas de decanulação. Método: Estudo retrospectivo de pacientes com menos de 18 anos submetidos a traqueostomia em um centro de saúde terciário, de janeiro de 2006 a novembro de 2015. Resultados: 123 crianças precisaram de uma traqueostomia após avaliação otorrinolaringológica durante o período do estudo. Do total, 63% eram do sexo masculino e 56% menores de um ano. Glossoptose foi a indicação mais comum (30%), seguida por estenose subglótica (16%) e faringomalácia (11%). A taxa de mortalidade foi de 31%. Até o fim deste artigo, 35 crianças (28,4%) haviam sido decanuladas e quanto menor o número de comorbidades, maior foi a taxa de decanulação (0,77 ± 0,84 vs. 1,7 ± 1,00 comorbidades; p < 0,001). Conclusão: A traqueostomia em crianças é um procedimento relativamente frequente em nosso hospital. As indicações mais comuns são glossoptose e estenose subglótica. Uma alta taxa de mortalidade foi encontrada, potencialmente comprovada pelo elevado número de pacientes críticos com condições neurológicas crônicas nessa coorte. Nossa taxa de decanulação está ligeiramente abaixo de outras séries, provavelmente por causa da maior quantidade de pacientes com comorbidades.

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PURPOSE: Newborns with Pierre Robin sequence (PRS) and syndromic micrognathia show microgenia and glossoptosis, which cause reduction of the airway and breathing difficulty from birth. Our goal is to analyze quantitative and qualitative volumetric changes before and after fast and early mandibular osteodistraction (FEMOD) and to compare radiological data. METHODS: The sample was composed of 4 patients, who satisfied inclusion criteria for completeness of data. Computed tomography pre- and post-operation were performed, then a volumetric assessment was made with Dolphin Imaging. Polysomnography was performed before and after FEMOD. RESULTS: Pre- and post-operative CT scan data were compared. The analysis of all three sections showed a significant increase of volumetric parameters. The retroglossal volume average increase was 346%, and the retropalatal volume average increase was 169%. These data matched the improvement recorded by polysomnography. CONCLUSIONS: The data confirm FEMOD as an efficient treatment to improve airways and breathing problem in patients affected by Pierre Robin sequence and syndromic micrognathia. The three-dimensional volume rendering could be a useful method to evaluate and quantify the increase in airways volume.

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INTRODUCTION: Children may require tracheostomy due to many different health conditions. Over the last 40 years, indications of tracheostomy have endorsed substantial modifications. OBJECTIVE: To evaluate pediatric patients warranted tracheostomy at our Hospital, in regard to their indications, associated comorbidities, complications and decannulation rates. METHODS: Retrospective study concerning patients under 18 years of age undergoing tracheostomy in a tertiary health care center, from January 2006 to November 2015. RESULTS: 123 children required a tracheostomy after ENT evaluation during the study period. A proportion of 63% was male, and 56% was under one year of age. Glossoptosis was the most common indication (30%), followed by subglottic stenosis (16%) and pharyngomalacia (11%). The mortality rate was 31%. By the end of this review, 35 children (28.4%) had been decannulated, and the fewer the number of comorbidities, the greater the decannulation rate (0.77±0.84 vs. 1.7±1.00 comorbidities; p<0.001). CONCLUSION: Tracheostomy in children is a relatively frequent procedure at our hospital. The most common indications are glossoptosis and subglottic stenosis. A high mortality rate was found, potentially substantiated by the high number of critical care patients with chronic neurological conditions in this cohort. Our decannulation rate is slightly below other series, probably because of the greater amount of patients with comorbidities.

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Pierre Robin sequence (formerly a syndrome) is named after the French stomatologist who, in 1923 and 1934, described the problems associated with micrognathia in newborn. It comprises mandibular micrognathia, U-shaped cleft palate, and glossoptosis. The typical symptoms are hypoxaemia, noisy breathing, snoring, stridor, cyanosis, bradycardia, feeding difficulties, and failure to thrive. Distraction osteogenesis has recently been considered as a surgical option for early intervention to lengthen the mandible and relieve respiratory problems. Piezosurgery offers a modality to make precise bone cuts preserving vital structures, minimizing the invasiveness of the surgical procedure, and offering bloodless field. We present case of 1-year-old male malnourished child with Pierre Robin sequence and tracheostomy in situ since day 11 of his birth. The staged treatment plan involving mandibular lengthening in which mandibular osteotomies were performed with the piezoelectric scalpel followed by decannulation of tracheostomy, which has been described in detail in this article.

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Pierre Robin Sequence is a congenital pathology defined by the triad micrognathia, glossoptosis and often a U-shaped cleft of soft palate. Newborns affected by airways obstruction may necessitate more invasive options: tongue-lip adhesion, tracheostomy and mandibular distraction osteogenesis. The authors analyzed the effect of fast and early mandibular osteodistraction on deciduous dental development in patients affected by Pierre Robin Sequence. Analysis of the patients treated for severe form was performed by a team composed by maxillofacial surgeons and dentists. Five patients were included for the analysis: before and long term clinical and radiological assessments were considered. All patients underwent fast and early mandibular osteodistraction; two years follow up computed tomography and panorex reconstructions showed bone consolidation, 33 of 35 teeth analyzed before ostedistraction are present after distraction protocol; no positional changes were detected at the follow up analysis either deciduous teeth and molar permanent buds. No deformities regarding molar buds were detected. In conclusion external mandibular distractor devices have been associated with dental injuries and facial scaring. Even though, the dental complications identified can not be unambiguously connected to the external distractor devices.

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BACKGROUND: Robin sequence (RS) is defined as a triad of retrognathia, glossoptosis, and airway obstruction. Although several studies have reported on the efficacy of mandibular distraction, the risks associated with this operation remain unclear. An outcomes analysis focusing on complications is reported here. METHODS: A 7-year retrospective review of all patients with RS treated with mandibular distraction was performed. Recorded variables included associated medical comorbidities, improvement in apnea/hypopnea index, need for tracheostomy, repeat distraction, and complications. Complications associated with mandibular distraction were categorized as major, moderate, or minor. They included surgical site infection (SSI), device failure, temporomandibular joint ankylosis, facial nerve injury, hypertrophic scarring, self-extubation premature ossification, and fibrous nonunion. RESULTS: Fifty patients were identified. Four patients (8%) required tracheostomy following distraction, and 3 required repeat distraction. There were 0% major, 12% moderate, and 18% minor complications. Moderate complications were device failure (2%), SSI requiring return to the operating room (2%), and SSI requiring intravenous antibiotics (8%). Minor complications were SSI managed with oral antibiotics (12%), self-extubation (4%), and transient facial nerve palsy (2%). The most common complication was SSI (22%), of which 90.9% were successfully treated by antibiotics alone. There was a 0% rate of temporomandibular joint ankylosis. CONCLUSIONS: Mandibular distraction is a safe and effective treatment option for infants with RS and severe airway obstruction. The most common complication is infection; the majority of cases are successfully treated with antibiotics alone.

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Assessing the severity of glossopharyngeal obstruction is important for the diagnosis and therapy of obstructive sleep apnea hypopnea syndrome (OSAHS). The polysomnography (PSG) with nasopharyngeal tube insertion (NPT-PSG) has shown good results in assessing glossopharyngeal obstruction. The objective of this study was to compare NPT-PSG with Friedman tongue position (FTP) classification which was also used to evaluate the glossopharyngeal obstruction. One hundred and five patients with OSAHS diagnosed by PSG were included in the study. All the patients were successfully examined by NPT-PSG. Based on the grade of FTP classification, 105 patients were divided into four groups. The differences of the general clinical data, PSG and NPT-PSG results were analyzed among the four groups. And the coincidence of diagnosing glossopharyngeal obstruction of two methods was calculated. There was no significant difference among the four groups in general clinical data and PSG results. However, NPT-PSG results were significantly different among the four groups. Following with the increasing FTP, apnea hypopnea index increased and lowest saturation of blood oxygen decreased. There were 38 patients with and other 38 patients without glossopharyngeal obstruction diagnosed by both methods. The coincidence of two methods was 72.4 %. NPT-PSG is an easy and effective method in assessing the severity of glossopharyngeal obstruction. The coincidence between the NPT-PSG and FTP classification is good. But in some special OSAHS patients such as glossoptosis, unsuccessful uvulopalatopharyngoplasty or suspicious pachyglossia, NPT-PSG is better than FTP classification.

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