RESUMO
MPZL2-related hearing loss is a rare form of autosomal recessive hearing loss characterized by progressive, mild sloping to severe sensorineural hearing loss. Thirty-five previously reported patients had biallelic truncating variants in MPZL2, with the exception of one patient with a missense variant of uncertain significance and a truncating variant. Here, we describe the clinical characteristics and genotypes of five patients from four families with confirmed MPZL2-related hearing loss. A rare missense likely pathogenic variant [NM_005797.4(MPZL2):c.280C>T,p.(Arg94Trp)] located in exon 3 was confirmed to be in trans with a recurrent pathogenic truncating variant that segregated with hearing loss in three of the patients from two unrelated families. This is the first recurrent likely pathogenic missense variant identified in MPZL2. Apparently milder or later-onset hearing loss associated with rare missense variants in MPZL2 indicates that some missense variants in this gene may cause a milder phenotype than that resulting from homozygous or compound heterozygous truncating variants. This study, along with the identification of truncating loss of function and missense MPZL2 variants in several diverse populations, suggests that MPZL2-related hearing loss may be more common than previously appreciated and demonstrates the need for MPZL2 inclusion in hearing loss testing panels.
Assuntos
Moléculas de Adesão Celular , Perda Auditiva Neurossensorial , Humanos , Moléculas de Adesão Celular/genética , Surdez/genética , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/patologia , Mutação de Sentido Incorreto/genética , Linhagem , FenótipoRESUMO
OBJECTIVES: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients. METHODS: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model. CONCLUSIONS: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD.
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Transtornos de Deglutição , Gastroenterologia , Medicina , Humanos , Criança , Transtornos de Deglutição/diagnóstico , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/terapia , PulmãoRESUMO
BACKGROUND: Ketorolac-refractory pain behavior following bilateral myringotomy and pressure equalization tube placement (BMT) is associated with the absence of middle ear fluid. Intraoperative fentanyl/ketorolac affords more reliable pain control than ketorolac alone. We hypothesized that middle ear condition would correlate with postoperative pain despite such combination therapy. We further sought to demonstrate seasonal variation in ear condition and its influence on pain. METHODS: We conducted a single-institution retrospective cohort study of healthy children (9 months-7 years), who underwent BMT by a single surgeon from 2015 to 2020. Anesthetic care included sevoflurane/nitrous oxide/oxygen/air by mask and intramuscular fentanyl/ketorolac. Left/right middle ear fluid status was recorded at the time of BMT, and ear condition (primary exposure) was dichotomized as bilateral infected (mucoid or purulent) or normal/unilateral infected. The primary outcome was maximum postanesthesia care unit Face, Legs, Activity, Cry, and Consolability (FLACC) score: 4-10 (moderate-to-severe pain) versus 0-3 (no-to-low pain). Rescue oxycodone, acetaminophen administration, and emergence agitation were secondary outcomes. Statistical analysis incorporated generalized linear mixed-effect models (GLMMs) with random intercepts to account for clustering by anesthesia provider. A year-over-year monthly time-series analysis was conducted using an autoregressive integrated moving average (ARIMA) regression model. RESULTS: Excluding recurrent cases, 1149 unique evaluable subjects remained. Bilateral infection prevalence was 39.8% (457/1149; 95% confidence interval [CI], 37.0-42.6). Probability of moderate-to-severe pain behavior was 23.5% (270/1149; 95% CI, 21.1-26.0) overall. Compared to patients with bilateral infected middle ears, those with normal/unilateral infected ears were more likely to have a FLACC score ≥4 (26.7% [185/692] versus 18.6% [85/457]; odds ratio [95% CI], 1.7 [1.2-2.3]; P = .002). Variability in pain outcome explained by the multivariable GLMM was 4.7%. Fentanyl dose response was evidenced by oxycodone administration differences ( P ≤ 0.002). Moderate-to-severe pain and emergence agitation were more likely with reduced fentanyl dosing. Bilateral infection prevalence exhibited seasonality, peaking in March and nadiring in July. However, pain outcomes did not vary by season. CONCLUSIONS: Normal/unilateral infected ears at time of pediatric BMT are associated with higher incidence of moderate-to-severe postoperative pain following intraoperative fentanyl/ketorolac administration, but the predictive value of ear condition on pain is limited. Infections were less common in the summer.
Assuntos
Delírio do Despertar , Cetorolaco , Humanos , Criança , Fentanila , Estações do Ano , Oxicodona/uso terapêutico , Estudos Retrospectivos , Dor Pós-Operatória/diagnóstico , Dor Pós-Operatória/tratamento farmacológico , Dor Pós-Operatória/epidemiologia , Orelha Média/cirurgia , Método Duplo-CegoRESUMO
OBJECTIVES: Based on an interpretation of a recent Joint Commission protocol to sterilize instruments coming into contact with mucous membranes, there has been institutional interest in sterile packaging of cerumen curettes used for manual cerumen removal. To date, there are no studies that have assessed the risk of otitis externa (OE) following cerumen removal and the utility of sterile packaging is questionable. The objective of this study is to assess the incidence of OE following cerumen disimpaction prior to the implementation of sterile packaging at our institution. METHODS: This was a retrospective chart review. Over a I-year period, 1457 episodes of manual cerumen removal took place in the otolaryngology clinic. Charts were assessed for signs or symptoms of OE within 2 weeks of the procedure through follow-up phone calls and clinic visits in the otolaryngology division. RESULTS: There were no patients who followed up with symptoms or signs suggestive of OE in the 2-week postprocedure period. CONCLUSION: There is no evidence that OE is a complication of manual cerumen removal when performed by otolaryngologists using clean technique. Unnecessary sterilization of tools leads to increased cost and time for this common outpatient procedure performed by the otolaryngologist.
Assuntos
Cerume , Curetagem/efeitos adversos , Desbridamento/efeitos adversos , Otite Externa/etiologia , Adolescente , Criança , Pré-Escolar , Curetagem/instrumentação , Desbridamento/instrumentação , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Medição de Risco , EsterilizaçãoRESUMO
OBJECTIVE: To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS: Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS: Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION: Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.
Assuntos
Otolaringologia , Lactente , Criança , Humanos , Consenso , Inquéritos e Questionários , Técnica DelphiRESUMO
OBJECTIVES: We review and stage a series of congenital lymphatic malformations (LMs) that caused laryngeal obstruction according to a proposed staging system, review the treatment modalities utilized, and correlate the stage of disease with clinical outcomes and tracheotomy statuses. METHODS: We present a detailed description of the clinical presentation, management approach, and clinical outcome and tracheotomy status of a series of patients with laryngeal obstruction by LMs. A 4-part staging system (stages I to IV) for congenital LMs obstructing the larynx, based upon our clinical experience, is proposed. The patients were retrospectively reviewed and staged according to the proposed system, and clinical management and outcomes were correlated with the stage of disease. RESULTS: We identified 16 patients with laryngeal obstruction by LMs. Eighty-one percent (13 of 16) received sclerotherapy, and 50% (8 of 16) underwent operative excision or debridement. Forty percent (2 of 5) of stage I lesions, 75% (3 of 4) of stage II lesions, 100% (4 of 4) of stage III lesions, and 100% (3 of 3) of stage IV lesions were in patients who required tracheotomy. All patients who had stage I and II lesions and required tracheotomy have been decannulated, whereas only 2 of the 4 patients with stage III lesions and no patients with stage IV lesions have been successfully decannulated. CONCLUSIONS: Lymphatic malformations obstructing the larynx require a careful and often staged management approach. A proposed staging system helps to predict the need for tracheotomy and the likelihood of long-term tracheotomy dependence.
Assuntos
Laringoestenose/etiologia , Laringe/anormalidades , Anormalidades Linfáticas/complicações , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Laringoscopia , Laringoestenose/diagnóstico , Laringoestenose/terapia , Anormalidades Linfáticas/diagnóstico , Masculino , Pescoço , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVES: Tracheocutaneous fistula (TCF) is a common occurrence after pediatric tracheostomy decannulation. However, the persistence of TCF after staged reconstruction of the pediatric airway is not well-described. The primary objective was to determine the rate of persistent TCF after successful decannulation in children with staged open airway reconstruction. METHODS: A case series with chart review of children who underwent decannulation after double-stage laryngotracheal reconstruction between 2017 and 2019. RESULTS: A total of 26 children were included. The most common open airway procedure was anterior and posterior costal cartilage grafting (84.6%, 22/26). Median age at decannulation was 3.4 years (IQR: 2.8-4.3) and occurred 7.0 months (IQR: 4.3-10.4) after airway reconstruction. TCF persisted in 84.6% (22/26) of children while 15.4% (4/26) of stomas closed spontaneously. All closures were identified by the one-month follow-up visit. There was no difference in age at tracheostomy (P = .86), age at decannulation (P = .97), duration of tracheostomy (P = .43), or gestational age (P = .23) between stomas that persisted or closed. Median diameter of stent used at reconstruction was larger in TCFs that persisted (7.0 mm vs 6.5 mm, P = .03). Tracheostomy tube diameter (P = .02) and stent size (P < .01) correlated with persistence of TCF on multivariable logistic regression analysis. There were 16 surgical closure procedures, which occurred at a median of 14.4 months (IQR: 11.4-15.4) after decannulation. Techniques included 56.3% (9/16) by primary closure, 18.8% (3/16) by secondary intention and 25% (4/16) by cartilage tracheoplasty. The overall success of closure was 93.8% (15/16) at latest follow-up. CONCLUSIONS: Persistent TCF occurs in 85% of children who are successfully decannulated after staged open airway reconstruction. Spontaneous closure could be identified by 1 month after decannulation and was more likely when smaller stents and tracheostomy tubes were utilized. Surgeons should counsel families on the frequency of TCF and the potential for additional procedures needed for closure.
Assuntos
Fístula Cutânea/epidemiologia , Laringoestenose/cirurgia , Procedimentos de Cirurgia Plástica/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Doenças da Traqueia/epidemiologia , Traqueostomia/efeitos adversos , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVE: The first pediatric tracheostomy tube change often occurs within 7 days after placement; however, the optimal timing is not known. The primary objective was to determine the rate of adverse events of an early tube change. Secondary objectives compared rates of significant peristomal wounds, sedation requirements, and expedited intensive care discharges. STUDY DESIGN: Prospective randomized controlled trial. SETTING: Tertiary children's hospital between October 2018 and April 2020. METHODS: A randomized controlled trial enrolled children under 24 months to early (day 4) or late (day 7) first tracheostomy tube changes. RESULTS: Sixteen children were enrolled with 10 randomized to an early change. Median age was 5.9 months (interquartile range, 5.4-8.3), and 86.7% required tracheostomy for respiratory failure. All tracheostomy tube changes were performed without adverse events. There were no accidental decannulations. Significant wounds developed in 10% of children with early tracheostomy tube changes and 83.3% of children with late tracheostomy tube changes (odds ratio [OR], 45.0; 95% CI, 2.3-885.6; P = .01). This significant reduction in wound complications justified concluding trial enrollment. Hours of dexmedetomidine sedation (P = .11) and boluses of midazolam during the first 7 days (P = .08) were no different between groups. After the first change, 90% of the early group were discharged from intensive care within 5 weeks compared to 33.3% of patients in the late group (OR, 18.0; 95% CI, 1.2-260.9; P = .03). CONCLUSION: The first tracheostomy tube change in children can occur without adverse events on day 4, resulting in fewer significant peristomal wounds and earlier intensive care discharge.
Assuntos
Traqueostomia/instrumentação , Traqueostomia/métodos , Feminino , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Fatores de TempoRESUMO
OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. STUDY DESIGN: Blinded modified Delphi consensus process. SETTING: Tertiary care center. METHODS: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. RESULTS: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. CONCLUSIONS: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:1168-1174, 2021.
Assuntos
Competência Clínica/normas , Consenso , Esofagoscopia/educação , Internato e Residência/normas , Cirurgiões/normas , Criança , Técnica Delphi , Esofagoscópios , Esofagoscopia/instrumentação , Esôfago/diagnóstico por imagem , Esôfago/cirurgia , Corpos Estranhos/diagnóstico , Corpos Estranhos/cirurgia , Humanos , Cirurgiões/educação , Cirurgiões/estatística & dados numéricos , Inquéritos e Questionários/estatística & dados numéricosRESUMO
OBJECTIVE: Intracranial complications of sinusitis (ICS) are uncommon in the antibiotic era. The role of neurosurgical drainage of ICS is fairly well established, but the role for acute surgical intervention for the sinus disease is not well defined. We retrospectively reviewed our experience with ICS to see if we could identify the role and effectiveness of endoscopic sinus surgery (ESS) in the acute setting of ICS. METHODS: The study used a retrospective review of patients presenting to a tertiary care academic medical center for a 6-year period. RESULTS: Twenty-three patients were identified with ICS, including epidural (8), subdural (10), intracerebral abscesses (2), and meningitis (3). Males were more affected than females (7:1). Twenty patients were 21 years old or younger. Twenty-two patients (96%) had radiologic evidence of frontal sinusitis with prefrontal or frontal lobe ICS at presentation. Medical therapy alone was successful in avoiding craniotomy in only 3 of 8 cases. Endoscopic sinus surgery and intravenous antibiotics as initial treatment was successful in avoiding craniotomy in only 1 of 6 patients. Of 23 patients, 18 underwent neurosurgical procedures--9 emergent procedures for abscesses more than 1 cm and 9 delayed procedures for persistent disease despite ICS less than 1 cm at presentation. CONCLUSIONS: Intracranial complications of sinusitis usually result from indirect spread of acute frontal sinusitis. The role of ESS in the initial treatment of ICS is not clear. In our series, ESS did not appear to alter the need for neurosurgical intervention, which was ultimately necessary in most patients with ICS, even with lesions less than 1 cm.
Assuntos
Abscesso Encefálico/etiologia , Endoscopia , Meningite/etiologia , Sinusite/complicações , Sinusite/cirurgia , Doença Aguda , Adolescente , Antibacterianos/uso terapêutico , Abscesso Encefálico/tratamento farmacológico , Abscesso Encefálico/cirurgia , Criança , Estudos de Coortes , Craniotomia , Drenagem , Feminino , Humanos , Masculino , Meningite/tratamento farmacológico , Meningite/cirurgia , Estudos Retrospectivos , Sinusite/tratamento farmacológico , Resultado do Tratamento , Adulto JovemRESUMO
Although the majority of attention to the health care impact of COVID-19 has focused on adult first responders and critical care providers, the pandemic has had a profound effect on the entire health care industry, including the pediatric otolaryngology community. This article highlights the unique ramifications of COVID-19 on pediatric otolaryngology, with a focus on the immediate and potential long-term shifts in practice. Specifically, the article is divided into 3 sections (care for the patient, care for the practitioner, and care for the practice) and details the unique effects of the pandemic on the pediatric otolaryngology specialty.
Assuntos
Infecções por Coronavirus/prevenção & controle , Procedimentos Cirúrgicos Eletivos/estatística & dados numéricos , Controle de Infecções/métodos , Procedimentos Cirúrgicos Otorrinolaringológicos/estatística & dados numéricos , Pandemias/prevenção & controle , Pneumonia Viral/prevenção & controle , Gestão da Segurança , COVID-19 , Criança , Pré-Escolar , Infecções por Coronavirus/epidemiologia , Procedimentos Cirúrgicos Eletivos/métodos , Feminino , Humanos , Masculino , Saúde Ocupacional , Procedimentos Cirúrgicos Otorrinolaringológicos/métodos , Pandemias/estatística & dados numéricos , Segurança do Paciente , Pediatria/métodos , Pneumonia Viral/epidemiologia , Estados UnidosRESUMO
INTRODUCTION: An airway assessment often occurs prior to tracheocutaneous fistula (TCF) closure in children. Bronchoscopy (MLB) with or without fistula-occluded polysomnography (PSG) helps determine candidacy and localize potential obstruction. To date, little has been published on MLB or PSG findings in children before surgically closing a TCF. METHODS: A case series with chart review of children between 2017 and 2020 who underwent repair of a TCF after tracheostomy decannulation. RESULTS: Thirty-six children were included for review. Mean age was 5.9 years (95% CI: 4.5-7.3), 58.3% were male, and 50% had chronic lung disease. Surgery occurred 13.3 months (95% CI: 11.9-14.8) after decannulation, with 80.6% by primary closure and 19.4% by secondary intention. There was one unsuccessful closure and two patients (5.6%) presented with a postoperative complication. An MLB was performed in 97.2% of children, where 22.9% identified supraglottic pathology, 11.4% had grade 2 subglottic stenosis, and 11.4% had difficult exposure of the larynx. Further, one child had a non-obstructing subglottic cyst, one had a supraglottoplasty for redundant arytenoid mucosa, and two children had suprastomal granulomas requiring removal. A PSG was obtained in 36.1%, with a mean Apnea-Hypopnea Index of 2.4 events/hour (95% CI: 0.9-3.9), nadir Oxygen saturation of 90.5% (95% CI: 87.9-93.0), and peak end-tidal CO2 of 46.1 mmHg (95% CI: 43.7-48.5). CONCLUSION: The selection of candidates for pediatric TCF closure requires careful evaluation of the airway. Surgeons should be familiar with the potential findings on MLB and PSG prior to closure.
Assuntos
Broncoscopia , Seleção de Pacientes , Polissonografia , Ferida Cirúrgica/cirurgia , Traqueostomia , Criança , Pré-Escolar , Feminino , Humanos , Doenças da Laringe/complicações , Doenças da Laringe/diagnóstico por imagem , Masculino , Oxigênio/sangue , Complicações Pós-Operatórias/etiologia , Período Pré-Operatório , Estudos Retrospectivos , Ferida Cirúrgica/complicaçõesRESUMO
OBJECTIVES: Many infants in the neonatal intensive care unit (NICU) require prolonged periods of respiratory support. Microlaryngoscopy and bronchoscopy (MLB) is performed to evaluate for airway pathology and facilitate decision-making regarding further airway interventions or tracheostomy. The objectives of this study are to describe the operative findings of MLB performed on infants in the NICU and determine which pre-operative characteristics or operative findings are predictive of the need for tracheostomy. METHODS: The medical records of preterm inpatients in the NICU at a single tertiary care hospital who underwent MLB between January 1, 2013 and January 7, 2016 were reviewed. Baseline and demographic characteristics and intra-operative findings were compared between patients who underwent tracheostomy and those who were successfully weaned from respiratory support. RESULTS: Seventy-three preterm patients underwent MLB for respiratory failure, of whom 41 (56.2%) underwent tracheostomy. Patients who underwent tracheostomy had lower mean gestational age (27.4 vs. 30.5 weeks), higher prevalence of bronchopulmonary dysplasia (73.2% vs. 37.5%), lower mean birth weight (1.1 kg vs. 1.6 kg), and a greater number of extubation events (5.2 vs. 3.0) than those who weaned from respiratory support. Abnormal MLB findings were common in both groups, though no single MLB finding differed significantly between groups. CONCLUSIONS: Preterm infants in the NICU with gestational age ≤30 weeks, birth weight <1.5 kg, severe pulmonary disease, and who have failed more than 3 extubation attempts are more likely to require tracheostomy.
Assuntos
Peso ao Nascer , Idade Gestacional , Insuficiência Respiratória/cirurgia , Traqueostomia , Extubação , Displasia Broncopulmonar/complicações , Broncoscopia , Feminino , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Unidades de Terapia Intensiva Neonatal , Laringoscopia , Masculino , Insuficiência Respiratória/complicações , Fatores de RiscoRESUMO
OBJECTIVE: Determine preoperative comorbidities and intraoperative parameters associated with adverse postoperative outcomes. METHODS: Retrospective case series at a single tertiary care children's hospital from 2010 through 2017. RESULTS: Twenty-six patients with median age of 6 months and median weight of 7.1 kg underwent slide tracheoplasty. Median time to extubation, length of intensive care unit admission, and length of hospitalization were 7, 27, and 30 days, respectively. Twenty-two (85%) required no additional intervention. Overall success was 87%. One (4%) patient required open revision, and 3 (11%) required tracheostomy. Concomitant cardiac surgery was associated with postoperative tracheostomy (p = 0.04). Age and weight at surgery were inversely correlated with length of intubation (p = 0.03) and length of hospital stay (p = 0.001, p = 0.002) respectively. Hospital stay was 2.2 times longer if preoperative mechanical ventilation was required (p = 0.01) and 39% longer for every 1 mm decrease in airway diameter at the narrowest portion of the stenosis (p = 0.005). There were no deaths related to persistent tracheal stenosis with a median follow-up of 24 months. CONCLUSION: Slide tracheoplasty is safe and effective. Concomitant cardiac surgery was associated with postoperative tracheostomy. Lower age and weight at surgery were correlated with longer length of intubation and hospital stay. Preoperative mechanical ventilation and smaller airway diameter were associated with longer hospital stay. This information may be helpful in counseling families and planning future prospective studies.
Assuntos
Procedimentos de Cirurgia Plástica , Estenose Traqueal/cirurgia , Extubação , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Tempo de Internação , Masculino , Respiração Artificial , Estudos Retrospectivos , Traqueostomia , Resultado do TratamentoRESUMO
OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.
Assuntos
Competência Clínica/normas , Pediatria/normas , Cirurgiões/normas , Traqueotomia/normas , Criança , Consenso , Técnica Delphi , Humanos , Pediatria/educação , Pediatria/métodos , Método Simples-Cego , Cirurgiões/educação , Traqueotomia/educaçãoRESUMO
BACKGROUND: Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. METHODS: A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. RESULTS: We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. CONCLUSION: In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.
Assuntos
Papillomavirus Humano 11/fisiologia , Papillomavirus Humano 6/fisiologia , Infecções por Papillomavirus/diagnóstico , Infecções por Papillomavirus/virologia , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/virologia , Adulto , Fatores Etários , Pré-Escolar , Condiloma Acuminado/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Mães , Infecções por Papillomavirus/epidemiologia , Infecções por Papillomavirus/cirurgia , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/cirurgiaRESUMO
Infections of the upper airways are a frequent cause of morbidity in children. Viral laryngotracheobronchitis (croup) is the most common cause of stridor in children and usually has a self-limited course with occasional relapses in early childhood. Epiglottitis has become rare in developed countries with the advent of universal vaccinations against Haemophilus influenzae. It can be rapidly fatal, however, if not promptly recognized and appropriately managed. This article reviews the pathogenesis, epidemiology, clinical presentation, diagnosis, and management of these pediatric upper airway infections.
Assuntos
Crupe/fisiopatologia , Crupe/virologia , Epiglotite/fisiopatologia , Epiglotite/virologia , Fatores Etários , Criança , Crupe/diagnóstico , Diagnóstico Diferencial , Epiglotite/diagnóstico , Humanos , Lactente , Síndrome do Desconforto Respiratório/diagnóstico , Sons RespiratóriosRESUMO
Laryngotracheoesophageal cleft (LTEC) is a rare anatomic cause of aspiration in the newborn and infant. The clinical presentation of infants with LTEC is variable and will usually vary with the degree of anatomic communication between the airway and esophagus. The diagnosis of LTEC is often challenging, particularly in cases where the presentation and pathology are more subtle. Management of LTEC depends upon the clinical presentation, degree of aspiration, and anatomic depth of the cleft. The goal of this review is to present the clinician with a practical approach to diagnosis and management of this rare but challenging congenital airway anomaly.
Assuntos
Anormalidades Múltiplas/cirurgia , Anormalidades Congênitas/diagnóstico , Esôfago/anormalidades , Laringe/anormalidades , Pneumonia Aspirativa/fisiopatologia , Traqueia/anormalidades , Anormalidades Múltiplas/diagnóstico , Anormalidades Congênitas/fisiopatologia , Anormalidades Congênitas/cirurgia , Esôfago/cirurgia , Humanos , Laringe/fisiopatologia , Laringe/cirurgia , Pneumonia Aspirativa/etiologia , Pneumonia Aspirativa/cirurgia , Guias de Prática Clínica como Assunto , Prognóstico , Traqueia/cirurgiaRESUMO
OBJECTIVE: To present our experience with balloon laryngoplasty (BL) as a means of establishing control of the compromised airway and as a definitive alternative to open surgery in infants with acquired subglottic stenosis (SGS). DESIGN: The medical charts of 10 consecutive infants diagnosed as having acquired SGS secondary to a history of intubation and treated initially with BL were reviewed. SETTING: Academic tertiary care children's hospital. PATIENTS: A total of 10 patients (3 girls and 7 boys), with a mean age of 4.8 months (range, 2-12 months), met the inclusion criteria for the study. MAIN OUTCOME MEASURES: The medical charts were assessed for the patients' demographics, clinical presentation, and outcomes, which were defined by postoperative symptomatology, endoscopic grading of residual SGS, complications, and the need for subsequent interventions to control SGS. RESULTS: All 10 patients presented with biphasic stridor, and 8 had significant retractions noted on examination. In all patients, control of the airway was established with BL followed by intubation. Four patients were completely asymptomatic after the initial BL. An additional 3 patients had recurrent stridor during the postoperative period and required a second BL before having complete, persistent resolution of symptoms. Balloon laryngoplasty failed in 3 patients, of whom 2 went on to undergo single-staged laryngotracheal reconstruction and 1 required a tracheotomy. CONCLUSIONS: Balloon laryngoplasty is a safe means of establishing the airway in infants with obstruction secondary to acquired SGS. It was an effective, stand-alone procedure for the management of SGS in 7 of our 10 patients, obviating the need for tracheotomy or cricoid split.