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1.
Georgian Med News ; (350): 57-62, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-39089272

RESUMO

The article provides a description of the clinical application of the authors' method of quantitative assessment of the degree of severity of glossoptosis based on clinical and X-ray anthropometric indicators (predictors). In the presentation of the scale for assessing clinical predictors (head position, shape of the palate, frenulum of the tongue, posture of the tongue by dr. John Mew, size of the palate), functional predictors (functional test with a bucket of water, index of degree of difficulty of tracheal intubation by dr. Seshagiri Rao Mallampati, degree of tongue elevation impairment for dr. S. Zaghi), X-ray - anthropometric indicators, (assessment of the position of the hyoid cyst along the C3-RGn line, type of slit growth). Each predictor was assessed by the number of scores. The sum of points according to all criteria indicated the level of importance of glossoptosis: mild severity - 0-20 points; moderate severity - 21-48 points; severe - 49 - 76 points. The algorithm for assessing the stage of glossoptosis is illustrated with a clinical case. The aim of the study - to improve the diagnosis of glossoptosis by determining clinical and X-ray anthropometric predictors of quantitative assessment of its severity. Results and discussion. As a result of the analysis of literature data and our own clinical studies, we have developed a methodology for the quantitative assessment of glossoptosis based on clinical and X-ray anthropometric predictors, namely: clinical predictors (head position, palate shape, tongue frenulum, tongue posture according to John Mew, the size of the palatine tonsils), functional predictors (functional test with a sip of water, the difficulty index of tracheal intubation according to Mallampati, the degree of tongue elevation according to S. Zaghi), X-ray - anthropometric predictors (estimation of the position of the hyoid bone relative to the C3-RGn line, type jaw growth) Conclusions. As a result of the analysis of special literature over the last 10 years and the conducted own clinical and additional examinations of 168 patients, a method of quantitative assessment of the severity of glossoptosis based on clinical and X-ray anthropometric indicators (predictors) was developed. It allows not only to detect the presence of glossoptosis, but also to quantitatively assess its severity: light - 0-20 points; average - 21-48 points; difficult - 49 - 76 points. What will allow us to plan orthodontic treatment, individualize myofunctional correction programs and motivate parents for successful cooperation with the doctor during the active period and during the retention period.


Assuntos
Glossoptose , Língua , Humanos , Língua/diagnóstico por imagem , Língua/patologia , Masculino , Glossoptose/diagnóstico por imagem , Glossoptose/patologia , Índice de Gravidade de Doença , Feminino , Antropometria/métodos , Radiografia , Adulto , Guias de Prática Clínica como Assunto
2.
Eur Arch Otorhinolaryngol ; 281(2): 1031-1039, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37975910

RESUMO

PURPOSE: To evaluate the percentage of obstructive sleep apnea (OSA) patients with retrolingual obstruction in all moderate-severe OSA patients and the proportions of different causes in all moderate-severe OSA patients with retrolingual obstruction and to discuss the accuracy of the Friedman tongue position (FTP) and retrolingual cross-sectional area (RCSA) in assessing the retrolingual obstruction. METHODS: Two hundred and twenty moderate-severe OSA patients were enrolled. After retrolingual obstruction was diagnosed, the percentage of OSA patients with retrolingual obstruction in all moderate-severe OSA patients was calculated. After that, the different causes of retrolingual obstruction were diagnosed based on different diagnostic criteria, and the proportions of different causes in all moderate-severe OSA patients with retrolingual obstruction were calculated. Finally, the correlations between FTP, RCSA, and apnea-hypopnea index after nasopharyngeal tube insertion (NPT-AHI) were analyzed, and the proportions of different causes of retrolingual obstruction based on different FTP and RCSA were observed. RESULTS: There were 128 patients with retrolingual obstruction, accounting for 58.2% of all moderate-severe OSA patients. In 128 patients with retrolingual obstruction, the proportions of glossoptosis (48.4%), palatal tonsil hypertrophy (28.1%), and lingual hypertrophy (8.6%) were relatively high. Both FTP and RCSA did not correlate with NPT-AHI. The proportion of lingual hypertrophy increased gradually with the increase of FTP and the proportions of glossoptosis in all FTP classifications were high. The patients with RCSA > 180 mm2 were mainly affected by glossoptosis and palatal tonsil hypertrophy, while patients with RCSA ≤ 180 mm2 were mainly affected by lingual hypertrophy. CONCLUSION: The percentage of patients with retrolingual obstruction in all moderate-severe OSA patients is relatively high, mainly glossoptosis, palatal tonsil hypertrophy, and lingual hypertrophy. FTP classification and RCSA can only reflect the retrolingual anatomical stenosis, but cannot fully reflect the retrolingual functional stenosis, especially the presence of glossoptosis.


Assuntos
Glossoptose , Apneia Obstrutiva do Sono , Humanos , Glossoptose/complicações , Constrição Patológica , Apneia Obstrutiva do Sono/complicações , Apneia Obstrutiva do Sono/diagnóstico , Nasofaringe , Hipertrofia/complicações
3.
J Perinatol ; 43(12): 1481-1485, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37816803

RESUMO

INTRODUCTION: The aim of the study is to evaluate major causes of upper airway obstruction in newborns receiving healthcare at our institution, their method of endoscopic assessment and the rate of complications related to these procedures. MATERIALS AND METHODS: This is a case series study of patients from institutional neonatal intensive care unit (NICU) presenting signs of ventilatory dysfunction for whom an endoscopic airway assessment was warranted. Information of interest was collected from medical records according to a Clinical and Endoscopic Assessment Protocol created for the study. The protocol included clinical manifestations needing ENT evaluation, clinical signs of ventilatory dysfunction, comorbidities (pulmonary, cardiac, neurological, and gastrointestinal), examination method (airway endoscopy under general anesthesia or awake), exam complications, and final diagnosis. RESULTS: One hundred sixty-nine newborn patients who underwent airway endoscopy (awake bedside flexible fiberoptic laryngoscopy (FFL) or direct laryngoscopy and bronchoscopy (DLB) in the surgical ward) were included. Thirty-nine patients (23.07%) underwent bedside FFL. For the remaining 130 who underwent DLB under general anesthesia, the median procedure time was 30 min (20-44). Only 9 (5.32%) patients presented complications: desaturation (4), laryngospasm without desaturation with spontaneous resolution (2), apnea with resolution after stimulation (1), seizures (1), nasal bleeding (1). The most frequent diagnoses found were glossoptosis, posterior laryngeal edema, and laryngomalacia. CONCLUSION: This retrospective case series describes the prevalence of different pathologies that cause upper airway obstruction in neonates. Airway endoscopy seems an effective and safe diagnostic tool in neonatal airway obstruction. Glossoptosis was the most prevalent cause of obstruction in our center.


Assuntos
Obstrução das Vias Respiratórias , Glossoptose , Humanos , Recém-Nascido , Lactente , Estudos Retrospectivos , Centros de Atenção Terciária , Glossoptose/complicações , Endoscopia , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Broncoscopia/efeitos adversos
4.
Int J Gynaecol Obstet ; 163(3): 778-781, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37231986

RESUMO

Treacher Collins syndrome (TCS) should be suspected if the triad of micrognathia, glossoptosis, and posterior cleft palate, and deformed external ears are observed during prenatal ultrasonography, excepting Pierre Robin sequence. Visualization of the fetal zygomatic bone and down-slanting palpebral fissures are conducive to differentiation. Molecular genetics testing can establish a definite diagnosis. A 28-year-old pregnant Chinese woman was referred for systematic ultrasound examination at 24 weeks. Two-dimensional and three-dimensional ultrasound showed polyhydramnios, micrognathia, absence of nasal bone, microtia, secondary cleft palate, mandibular hypoplasia, glossoptosis, and normal limbs and vertebrae. Pierre Robin sequence was misdiagnosed with the triad of micrognathia, glossoptosis, and posterior cleft palate. Final diagnosis of TCS was confirmed by whole-exome sequencing. Visualization of the fetal zygomatic bone and down-slanting palpebral fissures can facilitate a differential diagnosis between Pierre Robin sequence and TCS, with the triad of micrognathia, glossoptosis, and posterior cleft palate.


Assuntos
Fissura Palatina , Glossoptose , Disostose Mandibulofacial , Micrognatismo , Síndrome de Pierre Robin , Gravidez , Feminino , Humanos , Adulto , Disostose Mandibulofacial/diagnóstico por imagem , Disostose Mandibulofacial/genética , Síndrome de Pierre Robin/diagnóstico por imagem , Síndrome de Pierre Robin/genética , Micrognatismo/diagnóstico por imagem , Micrognatismo/genética , Glossoptose/complicações , Fissura Palatina/diagnóstico por imagem , Fissura Palatina/genética , Diagnóstico Pré-Natal
5.
Ann Plast Surg ; 90(5S Suppl 2): S221-S224, 2023 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-36752399

RESUMO

OBJECTIVE: This study aims to observe the safety and effectiveness of remimazolam benzenesulfonate combined with alfentanil for painless and comfort anesthesia in plastic surgery. METHODS: Two hundred patients with American Society of Anesthesiologists (ASA) I-II for elective facial autologous lipofilling + autologous liposuction (thigh or abdomen) plastic surgery in our hospital were selected. One hundred patients received comfort anesthesia (observation group) on odd-numbered day of surgery, and other 100 patients received painless anesthesia (control group) on even-numbered day. Patients in both groups were given slow injection of remimazolam benzenesulfonate 0.1 mg/kg and alfentanil 5 µg/kg to induce sleep before local anesthesia. Depending on body action reaction to surgical stimulation, patients in the observation group were received with remimazolam 0.05 mg/kg and alfentanil 2.5 µg/kg for maintenance until the end of surgery after local anesthesia, and patients in the control group received with remimazolam 0.25 to 0.5 mg/kg/h and alfentanil 25 to 50 µg/kg/h in continuous pumps. Time to fall asleep, sedation score, time to end of medication, time to open eyes, recovery score, and the presence of body movement, glossoptosis, arousal or jaw support during hypoxia, hypotension, bradycardia, operation time, total amount of remimazolam and alfentanil used, and the presence of postoperative complications such as pruritus, dizziness, nausea, and vomiting were recorded in both 2 groups. RESULTS: There were no significant differences in the preoperative vital sign parameters as mean arterial pressure, heart rate, and oxygen saturation between 2 groups ( P > 0.05). Intraoperative mean arterial pressure and heart rate were significantly lower in both groups compared with preoperative ( P < 0.05), but there was no statistically significant between the 2 groups ( P > 0.05). There was no significant decrease in oxygen saturation in both groups under the condition of intraoperative oxygen inhalation ( P > 0.05). There was no significant difference between the 2 groups in the incidence of adverse reactions, such as intraoperative body movement induced by skin cutting, glossoptosis requiring jaw thrust, postoperative pruritus, dizziness, nausea, and vomiting ( P > 0.05). There was no statistically significant difference in time to fall asleep, sedation score during local anesthesia, time to open eyes after stopping anesthetics, and recovery score between the 2 groups ( P > 0.05). Meanwhile, the total amount of remimazolam and alfentanil use was significantly reduced in patients in the observation group compared with the control group ( P < 0.05). CONCLUSIONS: Remimazolam benzenesulfonate combined with alfentanil can be used as a comfort anesthesia and painless anesthesia protocol in plastic surgery, which has the advantages of rapid onset of action, safety and comfort for patients, rapid recovery, and good cooperation. Furthermore, the protocol of remimazolam benzenesulfonate combined with alfentanil used in the observation group can significantly reduce the total amount of remimazolam and alfentanil used.


Assuntos
Glossoptose , Propofol , Cirurgia Plástica , Humanos , Alfentanil/efeitos adversos , Anestesia Local , Benzenossulfonatos , Tontura/induzido quimicamente , Náusea/induzido quimicamente , Propofol/efeitos adversos , Vômito/induzido quimicamente
6.
Cleft Palate Craniofac J ; 60(8): 986-992, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-35306875

RESUMO

OBJECTIVE: To determine the risk factors and their respective magnitudes for developing Obstructive Sleep Apnea (OSA) in Down syndrome (DS) patients. DESIGN: Retrospective cohort study. PATIENTS: The 2016 Kids' Inpatient Database (KID) was queried to identify all patients diagnosed with DS. MAIN OUTCOME MEASURES: The primary predictor variables were tonsillar hypertrophy (TH), adenoidal hypertrophy (AH), Hypertrophy of Tonsils & Adenoids (HTA), Laryngeal Stenosis (LS), Hypotonia, Glossoptosis, Congenital Laryngomalacia (CL), and Overweight & Obesity (OO). The primary outcome variable was OSA. RESULTS: The final sample consisted of 18,181 patients with a diagnosis of DS. Relative to patients aged 0-5, patients aged 6-10 (OR 3.5, P < 0.01), 11-5 (OR 3.4, P < 0.01), and 16 & above (OR 3.6, P < 0.01) were each independently associated with increased odds of OSA. Further, TH (OR 23.2, P < 0.01), AH (OR 20.3, P < 0.01), HTA (OR 64.2, P < 0.01), glossoptosis (OR 5.0, P < 0.01), CL (OR 4.3, P < 0.01), and OO (OR 3.7, P < 0.01) were all independent risk factors for OSA. CONCLUSIONS: The presence of hypertrophied tonsils and adenoids together was the strongest risk factor for OSA. DS patients aged six and above were at risk for OSA development relative to younger patients. Patients with DS should be tested for OSA, which otherwise will deteriorate their existing comorbidities.


Assuntos
Síndrome de Down , Glossoptose , Apneia Obstrutiva do Sono , Humanos , Síndrome de Down/complicações , Síndrome de Down/epidemiologia , Estudos Retrospectivos , Apneia Obstrutiva do Sono/epidemiologia , Apneia Obstrutiva do Sono/etiologia , Fatores de Risco , Hipertrofia/complicações
7.
J Hum Genet ; 67(11): 675-678, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35970985

RESUMO

Pierre-Robin sequence (PRS) is a rare, congenital defect presenting with micrognathia, glossoptosis, and airway obstruction with variable inclusion of a cleft palate. Overlapping PRS with neurofibromatosis type 2 (NF2) is a syndrome caused by a chromosome 22q12 microdeletion including NF2. We describe a patient with severe early-onset NF2 overlapping with PRS that showed micrognathia, glossoptosis, and a mild form of cleft palate. We detected a de novo chromosome 22q12 microdeletion including MN1 and NF2 in the patient. Previous cases of overlapping PRS and NF2 caused by the chromosome 22q12 microdeletions showed severe NF2 phenotypes with variable severity of cleft palate and microdeletions of varying sizes. Genotype-phenotype correlations and comparison of the size and breakpoint of microdeletions suggest that some modifier genes distal to MN1 and NF2 might be linked to the cleft palate severity.


Assuntos
Fissura Palatina , Glossoptose , Micrognatismo , Neurofibromatose 2 , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/genética , Neurofibromatose 2/genética , Fissura Palatina/genética , Micrognatismo/genética , Cromossomos , Transativadores/genética , Proteínas Supressoras de Tumor/genética
10.
Laryngoscope ; 131(12): 2811-2816, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34117782

RESUMO

OBJECTIVES/HYPOTHESIS: Robin sequence (RS) consists of associated micrognathia, glossoptosis, and respiratory dysfunction, with or without cleft palate. Studies on how different patient characteristics impact the severity of respiratory dysfunction are scarce and contradictory; this study investigates how different features affect respiratory obstruction severity at diagnosis of RS in controlled analysis. STUDY DESIGN: Retrospective cohort study that enrolled 71 RS patients under 90 days old who received care in our institution from 2009 to 2020. METHODS: The primary outcome, respiratory dysfunction, was categorized into four severity groups and analyzed using a multinomial logistic regression model that considered age, sex, mandible length, cleft palate, syndromic diagnosis, other airway anomalies, and degree of glossoptosis. RESULTS: Mandible length, syndromic diagnosis, and Yellon grade 3 glossoptosis were related to poorer respiratory outcomes (need for respiratory support). In univariate analysis, for each additional 1 mm of mandible length at diagnosis, a mean reduction of 28% in the risk of needing respiratory support was observed (OR = 0.72; 0.58-0.89); syndromic diagnosis and grade 3 glossoptosis also raised the risk (OR = 6.50; 1.59-26.51 and OR = 12.75; 1.03-157.14, respectively). In multivariate analysis, only mandible length significantly maintained its effects (OR = 0.73; 0.56-0.96), a 27% reduction. CONCLUSIONS: Mandible length was an independent predictor for more severe respiratory dysfunction in RS patients, with larger mandibles showing protective effects. Syndromic diagnosis and Yellon grade 3 glossoptosis are also likely to be associated with poorer respiratory outcomes, although this was not demonstrated in multivariate analysis. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2811-2816, 2021.


Assuntos
Glossoptose/complicações , Síndrome de Pierre Robin/complicações , Transtornos Respiratórios/epidemiologia , Feminino , Glossoptose/diagnóstico , Glossoptose/patologia , Humanos , Imageamento Tridimensional , Lactente , Recém-Nascido , Masculino , Mandíbula/diagnóstico por imagem , Mandíbula/patologia , Tamanho do Órgão , Síndrome de Pierre Robin/diagnóstico , Síndrome de Pierre Robin/patologia , Prognóstico , Fatores de Proteção , Transtornos Respiratórios/diagnóstico , Transtornos Respiratórios/etiologia , Estudos Retrospectivos , Medição de Risco/métodos , Medição de Risco/estatística & dados numéricos , Fatores de Risco , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X
12.
Metro cienc ; 29(1 (2021): Enero- Marzo): 44-50, 2021-01-29.
Artigo em Espanhol | LILACS | ID: biblio-1337679

RESUMO

La secuencia de Pierre Robin (SPR) es una enfermedad rara, caracterizada por una tríada de malformaciones orofaciales como retrognatia, glosoptosis y fisura velopalatina media (paladar hendido); las cuales, generalmente provocan obstrucción de la vía aérea (OVA). La corrección de los defectos palatinos requiere en ocasiones varias intervenciones quirúrgicas, por esta razón el cuidado anestésico y abordaje de la vía aérea en forma adecuada, evita complica-ciones asociadas a esta enfermedad. Se presenta el caso de un paciente de 1 año y 20 días de edad, con antecedentes de vía aérea difícil, intentos fallidos de intubaciones preliminares e intervenido quirúrgicamente para corrección de paladar hendido, luego de una técnica combinada de intubación nasal con fibrobroncoscopio, desplazamiento de glosoptosis con ayuda de pala número 2 de videolaringoscopio (Glidescope®); manejo transoperatorio anestésico y proceso de extubación con excelentes resultados. Destacamos la importancia de realizar una adecuada planificación multidisciplinaria prequirúrgica con valoración exhaustiva de la vía aérea (VA) por laringoscopía directa o fibrobroncospia flexible para conocer el sitio exacto de la obstrucción y evitar desen-laces adversos.


Pierre Robin Sequence (PRS) is a rare disease characterized by a triad of orofacial malformations such as retrognathia, glossoptosis and velopalatine fissure (cleft palate). The malformations in PRS can lead into airway obstruction. The correction of the palatal defects sometimes requires several surgical interven-tions. Proper anesthetic care and a carefully planned approach to the airway can avoid complications associated with this disease. We present the case of a 1 year and 20 days old patient, with history of difficult airway, unsuccessful prior attempts of intubation, who underwent surgery to correct a cleft palate. We used a combined approach that included nasal intubation technique with a fiberoptic bronchoscope aided with a number 2 video laryngoscope blade (Gli-descope®) for displacement of the glossoptosys. Details of the intraoperative anesthetic management and subsequent successful extubation are provided. We highlight the importance of developing an adequate preoperative multidisciplinary plan of action, after a careful and detailed evaluation of the airway with direct laryngoscopy or fiberoptic bronchoscopy in order to identify the exact location of the obstruction and avoid adverse outcomes.


Assuntos
Humanos , Masculino , Lactente , Síndrome de Pierre Robin , Fissura Palatina , Manuseio das Vias Aéreas , Glossoptose , Intubação , Laringoscopia
13.
Cleft Palate Craniofac J ; 58(8): 1012-1019, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33228397

RESUMO

OBJECTIVE: Micrognathia in connection with glossoptosis (called Robin sequence) may lead to life-threatening respiratory problems immediately after birth. An objective detection during prenatal routine ultrasound sonography is possible using an index that relates fetal lower jaw length to femur length or gestational age. The aim of this study was to test the method's sensitivity and specificity and to discuss its predictive power concerning neonatal respiratory insufficiency. DESIGN: Patients with subjectively identified suspicious signs in the sagittal profile view were included in the study: Two-dimensional serial ultrasound scans of their fetal mandible were used to measure the lower jaw lengths and compare them to predicted values according to an index, derived from 313 healthy fetuses. Follow-up data provided additional information on the clinical appearance of the newborns. RESULTS: The index showed a high sensitivity: 15 of the 16 cases with a micrognathia were correctly diagnosed (sensitivity of 93.75%). Follow-up data showed that newborns with similar index values differed in terms of their upper airway obstruction and treatment need. CONCLUSION: Fetal mandibular micrognathia can be objectively evaluated with the help of the index. The method allows an early detection of micrognathia, which helps to take the necessary steps for proper treatment of potential life-threatening respiratory impairment. Observations ranging outside the prediction interval could prompt the ultrasonographer to check for other associated malformations.


Assuntos
Obstrução das Vias Respiratórias , Glossoptose , Micrognatismo , Síndrome de Pierre Robin , Feminino , Humanos , Recém-Nascido , Mandíbula/diagnóstico por imagem , Micrognatismo/diagnóstico por imagem , Síndrome de Pierre Robin/diagnóstico por imagem , Gravidez , Diagnóstico Pré-Natal
14.
J Craniofac Surg ; 31(8): 2294-2296, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33136874

RESUMO

Previously, severe upper airway obstruction in patients with retrognathia and glossoptosis has been managed with tracheostomy. However, tracheostomy is associated with significant morbidity. In recent years, mandibular distraction has become an alternative management strategy in infants, but these applications have been limited to patient populations with retrognathia and glossoptosis. The authors present 2 unique cases of patients with KAT6B-related gene disorders, who present with a paradox of tongue-based airway obstruction in the absence of retrognathia. In both cases mandibular distraction osteogenesis with an obliquely oriented vector was successfully performed and both children avoided the need for tracheostomy.


Assuntos
Histona Acetiltransferases/genética , Doenças Mandibulares/genética , Osteogênese por Distração , Obstrução das Vias Respiratórias/cirurgia , Glossoptose , Humanos , Lactente , Recém-Nascido , Masculino , Doenças Mandibulares/cirurgia , Osteogênese por Distração/métodos , Retrognatismo/cirurgia , Traqueostomia
15.
Rev. bras. cir. plást ; 34(2): 228-236, apr.-jun. 2019. ilus, tab
Artigo em Inglês, Português | LILACS | ID: biblio-1015972

RESUMO

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.


Assuntos
Humanos , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicações , Osteogênese por Distração/métodos , Doenças do Nervo Glossofaríngeo/cirurgia , Doenças do Nervo Glossofaríngeo/complicações , Obstrução das Vias Respiratórias/cirurgia , Obstrução das Vias Respiratórias/complicações , Obstrução das Vias Respiratórias/diagnóstico , Glossoptose/cirurgia , Glossoptose/patologia , Micrognatismo/cirurgia , Micrognatismo/complicações , Soalho Bucal/anormalidades , Soalho Bucal/cirurgia
17.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-785807

RESUMO

Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. PRS does not have a single pathogenesis, but rather is associated with multiple syndromes. This report presents the case of a 35-year-old woman with PRS and scoliosis. Among the syndromes related to PRS, cerebro-costo-mandibular syndrome (CCMS), which is characterized by posterior rib gap defects and vertebral anomalies, was suspected in this patient. However, no posterior rib gap defect was detected on radiological examinations. Although over 80 cases of CCMS have been reported to date, few cases of PRS with scoliosis alone have been reported. Therefore, this report demonstrated the clinical, radiological, and cephalometric characteristics of an adult patient with PRS and scoliosis, but without rib anomalies.


Assuntos
Adulto , Feminino , Humanos , Obstrução das Vias Respiratórias , Glossoptose , Micrognatismo , Síndrome de Pierre Robin , Costelas , Escoliose
18.
Neonatal Medicine ; : 179-183, 2019.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-760582

RESUMO

Pierre Robin sequence (PRS), also called Robin sequence, is a congenital anomaly characterized by a triad of micrognathia, glossoptosis, and upper airway obstruction. Infants with PRS can present with varying degrees of respiratory difficulty secondary to upper airway obstruction. There has been no consensus for the treatment of upper airway obstruction in infants with PRS, but recent studies recommend attempting non-surgical interventions before surgical treatment. In this case report, we present 3 cases of infants diagnosed with PRS who showed persistent respiratory difficulties after birth. Before considering surgical intervention, insertion of a nasopharyngeal airway was attempted in these infants. Following this procedure, symptoms of upper airway obstruction were relieved, and all infants were discharged without surgical interventions; the nasopharyngeal airway was removed 1 to 2 months later. To date, no infant has shown signs of upper airway obstruction. Nasopharyngeal airway insertion is a highly effective and less invasive treatment option for infants with PRS. However, it is not widely known and used in Korea. Nasopharyngeal airway insertion can be preferentially considered before surgical intervention for upper airway obstruction in such infants.


Assuntos
Humanos , Lactente , Obstrução das Vias Respiratórias , Consenso , Glossoptose , Coreia (Geográfico) , Micrognatismo , Parto , Síndrome de Pierre Robin
19.
J Stomatol Oral Maxillofac Surg ; 119(5): 419-428, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29777780

RESUMO

Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form. Because of the clinical importance of PRS, it's fundamental to describe the features of the Robin sequence to clearly define its primary and secondary clinical signs useful to diagnosis. A complete evaluation of the syndrome allows choosing the most appropriate therapeutic treatment, opting for conservative or surgical management, in order to improve the quality of life of the patient.


Assuntos
Obstrução das Vias Respiratórias , Glossoptose , Micrognatismo , Síndrome de Pierre Robin , Humanos , Qualidade de Vida
20.
Cleft Palate Craniofac J ; 55(10): 1467-1469, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29617156

RESUMO

The presence of a bilobed tongue is a rare congenital malformation. There are multiple reports of a bifid anterior lobe; however, a case with complete separation of isolated anterior and posterior lobes has not been previously described. We report the case of a 2-year-old male, who presented with a V-shaped mandible and glossoptosis in the setting of respiratory distress and difficulty feeding, incidentally found to have a bilobed tongue with independent anterior and posterior lobes.


Assuntos
Glossoptose/congênito , Mandíbula/anormalidades , Língua/anormalidades , Humanos , Achados Incidentais , Lactente , Masculino , Síndrome do Desconforto Respiratório do Recém-Nascido/complicações
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