A traqueia é um tubo cartilaginoso que liga a laringe aos brônquios dos pulmões, permitindo a passagem do ar, e por isso está presente em quase todos os animais com pulmões. A traqueia se estende da laringe e se ramifica nos dois brônquios principais. No topo da traquéia, a cartilagem cricoide a liga à laringe. A traquéia é formada por vários anéis em forma de ferradura, unidos verticalmente por ligamentos sobrejacentes e pelo músculo traqueal em suas extremidades. A epiglote fecha a abertura da laringe durante a deglutição.
Introdução
O que você precisa saber de cara
Condição rara caracterizada pelo estreitamento da traqueia desde o nascimento, podendo causar dificuldade respiratória, choro fraco e sibilos. Frequentemente associada a outras anomalias congênitas, como as do trato gastrointestinal e cardíacas.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 15 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 36 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Estenose traqueal, congênita
Centros de Referência SUS
24 centros habilitados pelo SUS para Estenose traqueal, congênita
Centros para Estenose traqueal, congênita
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.
Partial anomalous left pulmonary artery sling is an exceptionally rare congenital anomaly, and its coexistence with ventricular septal defect and right lung agenesis has not been reported. We describe a neonate presenting with right lung agenesis, a large ventricular septal defect, partial anomalous left pulmonary artery sling, and congenital tracheal stenosis. Following surgical repair of the ventricular septal defect at 73 days of age, the patient showed marked improvement in respiratory status and was discharged home without respiratory support.
Unravelling the Complexity of Congenital Tracheal Stenosis with 'O' Rings.
Congenital tracheal stenosis (CTS) in paediatric patients is a rare condition, accounting for only 0.3% to 1% of all laryngotracheal stenoses. It is characterised by complete cartilaginous 'O'-shaped tracheal rings replacing the normal posterior membranous portion. This monocentric case series analyses management strategies and assesses surgical and functional outcomes. Twenty-one patients (12 males, 9 females) underwent surgery for CTS. Nineteen patients received slide tracheoplasty, while two underwent tracheal resection with primary anastomosis. Pre- and postoperative assessments focused on respiratory, swallowing, and vocal functions. The study also examined correlations between prior non-airway surgeries, extent of stenosis, and postoperative complications. All patients demonstrated postoperative improvement in respiratory, swallowing, and vocal functions. Wilcoxon signed-rank testing revealed a statistically significant improvement in respiratory scores, whereas improvements in voice scores did not reach statistical significance. Swallowing scores showed no variation pre- and postoperatively and were thus excluded from this analysis. A significant positive correlation was observed between cardiopulmonary bypass duration and postoperative intubation time (Pearson's r = 0.585, 95% CI 0.205-0.812, P = .007). In contrast, correlations between ICU length of stay and complication severity (Spearman's ρ = 0.316, 95% CI -0.113 to 0.787, P = .175) as well as between patient age at surgery and complication severity were not statistically significant. Additionally, linear associations were identified between prior non-airway surgeries and increased complication rates and between bypass time, hospital stay duration, and complication severity. Slide tracheoplasty remains the preferred technique for long-segment CTS, offering structural and functional restoration. Short-segment disease can be effectively managed with segmental resection and anastomosis. Prognosis could be influenced by stenosis length, associated comorbidities, and prior interventions, but larger studies would be needed to achieve adequate statistical power. Careful preoperative evaluation, tailored surgical planning, and attentive postoperative care are vital for optimal outcomes. Surgical intervention may be warranted even in select mildly symptomatic patients to prevent progression to life-threatening airway compromise.
Safety and efficacy of a helium-oxygen mix for pediatric respiratory failure associated with anatomical and functional stenotic lesions of the airway: a phase II clinical trial.
To evaluate the safety and efficacy of helium-oxygen mixture in Japanese pediatric patients with respiratory failure due to obstructive airway disease. This multicenter, single-arm, open-label trial was conducted at two tertiary pediatric hospitals in Japan. Patients aged <20 years requiring mechanical ventilation for bronchiolitis, congenital tracheal stenosis or asthma exacerbation were received helium-oxygen mixture via mechanical ventilation. The primary endpoint was the incidence of serious adverse events. The secondary endpoints included changes in airway resistance, partial pressure of arterial carbon dioxide (PaCO2), and tidal volume. Fifteen patients (46.7 % female; median age, 7 months) were enrolled with diagnosis of bronchiolitis (73.3 %), asthma (20.0 %), and congenital tracheal stenosis (6.7 %). No serious adverse events related to helium-oxygen mixture were observed (0 %; 95 % confidence interval [CI]: 0.0-21.8 %). Airway resistance decreased significantly from a baseline mean of 138.1 cm H2O/L/s to 90.7 cm H2O/L/s at 30 min after administration (mean change: -47.4; 95 % CI: -64.2 to -30.6) for an efficacy rate of 93.3 %. The changes in the PaCO2 and tidal volume were not statistically significant. The median time from helium-oxygen mixture administration to extubation was 25.6 h. Helium-oxygen mixture appeared to be safe, well tolerated, and was associated with a reduction in airway resistance in pediatric patients with obstructive airway disease. jRCT2091220357https://jrct.mhlw.go.jp/latest-detail/jRCT2091220357.
Biallelic TEDC1 variants cause a new syndrome with severe growth impairment and endocrine complications.
We encountered two affected male patients born to non-consanguineous parents, who presented with prenatal-onset severe growth impairment, primary microcephaly, developmental delay, adrenal insufficiency, congenital glaucoma, delayed bone aging, craniosynostosis, congenital tracheal stenosis, and primary hypogonadism. By exome sequencing, we identified compound heterozygous TEDC1 variants (NM_001134877.1 c.[104-5C>G];[787delG] p.[?];[(Ala263LeufsTer29)] in both affected siblings. We confirmed that the splice site variant, c.104-5C>G, leads to no TEDC1 protein production via nonsense-mediated mRNA decay. The frameshift variant located in the last coding exon, c.787delG, produces a C-terminally truncated protein, which impairs the binding with TEDC2. Thus, both variants are thought to be loss-of-function. TEDC1 and TEDC2 are both required for centriole stability and cell proliferation. Our in vitro experiments using patient-derived cells revealed cell cycle abnormality. Our in vivo study using tedc1-/- zebrafish generated by CRISPR/Cas9 successfully recapitulated the growth impairment and cranial bone dysplasia as seen in our patients. The tedc1-/- mutant zebrafish were sterile and did not have developed gonads. Furthermore, we showed that biallelic TEDC1 deletion causes cilia abnormalities through defective acetylated tubulins.
Surgical management of an ELBW neonatal PDA with congenital tracheal stenosis.
Publicações recentes
Computational fluid dynamics as an objective measure for assessing congenital tracheal stenosis: Proof-of-concept study.
Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.
From Childhood to Adulthood: A Case of Congenital Tracheal Stenosis.
Unravelling the Complexity of Congenital Tracheal Stenosis with 'O' Rings.
Safety and efficacy of a helium-oxygen mix for pediatric respiratory failure associated with anatomical and functional stenotic lesions of the airway: a phase II clinical trial.
📚 EuropePMC272 artigos no totalmostrando 171
Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.
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Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International ConferenceOutcome of congenital tracheal stenosis in children over two decades in a national cardiothoracic surgical unit.
Cardiology in the young[Congenital tracheal stenosis: from clinic to treatment.].
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International journal of pediatric otorhinolaryngologyNon-operative management of congenital tracheal stenosis: criteria by computed tomography.
Pediatric surgery internationalAsymptomatic congenital tracheal stenosis.
Respirology case reportsSpanish multicentre study on morbidity and pathogenicity of tracheal bronchus in children.
Pediatric pulmonologyExcessive Tracheal Length in Patients With Congenital Tracheal Stenosis.
The Annals of thoracic surgeryLong-term outcomes of surgery for pulmonary artery sling in children.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryA pilot study of a novel biodegradable magnesium alloy airway stent in a rabbit model.
International journal of pediatric otorhinolaryngologyTailored Management of Airway Abnormalities in Children with Congenital Single Functional Lung.
Journal of pediatric surgeryFluid dynamic assessment of tracheal flow in infants with congenital tracheal stenosis before and after surgery.
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Journal of cardiothoracic and vascular anesthesiaEnhanced chondrogenic differentiation of human mesenchymal stems cells on citric acid-modified chitosan hydrogel for tracheal cartilage regeneration applications.
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The LaryngoscopeAnesthetic Management for Concomitant Correction of Congenital Cardiac Defects and Long "O" Ring Tracheal Stenosis: A Role for Heliox?: Report of 2 Cases.
A&A practiceImpact of 3D printing on the surgical management of tracheal stenosis associated to pulmonary sling: a case report.
Journal of thoracic diseaseDifficult intubation due to unknown congenital tracheal stenosis in the adult: a case report and literature review.
Journal of thoracic diseaseSwallowing outcomes in children after slide tracheoplasty.
International journal of pediatric otorhinolaryngologyAn approach to successful slide tracheoplasty in the low birth weight neonate with single lung.
International journal of pediatric otorhinolaryngologyPediatric Interventional Pulmonology.
Clinics in chest medicineBronchial Mismatch as a Predictor of Respiratory Failure After Congenital Tracheal Stenosis Repair.
The Annals of thoracic surgeryClinical outcomes after tracheoplasty in patients with congenital tracheal stenosis in 1997-2014.
Journal of pediatric surgeryTo do or not to do? The management dilemma of congenital tracheal stenosis in the setting of the ring-sling complex.
Journal of thoracic diseaseLeft pulmonary artery sling and congenital tracheal stenosis: to slide or not to slide?
Journal of thoracic diseaseOperative or non-operative treatment of congenital tracheal stenosis: is there something new?
Journal of thoracic diseaseTracheal surgery in children: outcome of a 12-year survey.
Interactive cardiovascular and thoracic surgeryTemporary tracheal stenting using endovascular prosthesis in the management of severe refractory stenosis following slide tracheoplasty.
European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck SurgerySlide tracheoplasty in 81 children: Improved outcomes with modified surgical technique and optimal surgical age.
Medicine[Surgical treatment of tracheal stenosis. A 11-year report in a pediatric hospital].
Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia PediatricaNeonatal Tracheal and Intracardiac Repair in a High-Risk Premature Infant Requiring Preoperative ECMO Transport.
World journal for pediatric & congenital heart surgeryManagement of congenital tracheal stenosis in the neonatal period.
Pediatric surgery internationalAn Adolescent with Dyspnea and Cough. A Case of Congenital Tracheal Stenosis.
American journal of respiratory and critical care medicineAirway Problems in Neonates-A Review of the Current Investigation and Management Strategies.
Frontiers in pediatricsThe role of adjunctive procedures in reducing postoperative tracheobronchial obstruction in single lung patients with congenital tracheal stenosis undergoing slide tracheoplasty.
Journal of pediatric surgeryPediatric airway surgery.
Pediatric surgery internationalTracheal and bronchial stenoses and other obstructive conditions.
Journal of thoracic diseaseSlide tracheoplasty outcomes in children with congenital pulmonary malformations.
The LaryngoscopeBiodegradable polydioxanone stent as a new treatment strategy for tracheal stenosis in a rabbit model.
Journal of pediatric surgeryAdvances in Surgical Treatment of Congenital Airway Disease.
Seminars in thoracic and cardiovascular surgeryA case of pulmonary artery sling where onset Was induced by therapeutic hypothermia.
Pediatric pulmonologySurgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis.
Pediatric surgery internationalClinical equivalency of cardiopulmonary bypass and extracorporeal membrane oxygenation support for pediatric tracheal reconstruction.
Pediatric surgery internationalCongenital tracheobronchial stenosis.
Seminars in pediatric surgeryEmergency management for congenital tracheal stenosis with endoluminal stenting in pediatric intensive care units.
Therapeutic advances in respiratory diseaseThe association of congenital tracheobronchial stenosis and cardiovascular anomalies.
International journal of pediatric otorhinolaryngologyRapid Expansion of Human Epithelial Stem Cells Suitable for Airway Tissue Engineering.
American journal of respiratory and critical care medicineBridging bronchus, type six, as a new rare case of a bronchial anomaly.
JA clinical reportsEvolution of Surgical Approaches in the Management of Congenital Tracheal Stenosis: Single-Center Experience.
World journal for pediatric & congenital heart surgeryAbnormal cardiac axis as a prenatal marker of left pulmonary artery sling.
Pediatrics international : official journal of the Japan Pediatric SocietyTotal Airway Reconstruction in the Neonate: Combined Mandibular Distraction and Slide Tracheoplasty for Multiple Level Airway Obstruction.
The Journal of craniofacial surgery[Anesthesia Management for Primary Cleft Lip Closure in an Infant with Congenital Total Length Complete Tracheal Rings].
Masui. The Japanese journal of anesthesiologySurgical management of congenital tracheal stenosis associated with tracheal bronchus and congenital heart disease.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryManagement of Congenital Tracheal Stenosis.
Pediatrics[Advances in pediatric thoracic surgery during the past 50 years].
Nihon Geka Gakkai zasshiManagement of tracheobronchial obstruction in infants using metallic stents: long-term outcome.
Pediatric surgery internationalCongenital tracheal stenosis: unanticipated and anticipated difficult airway management in a neonate.
A & A case reportsModified end-to-end anastomosis for the treatment of congenital tracheal stenosis with a bridging bronchus.
The Annals of thoracic surgeryAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.
- Unravelling the Complexity of Congenital Tracheal Stenosis with 'O' Rings.European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery· 2026· PMID 41416901mais citado
- Safety and efficacy of a helium-oxygen mix for pediatric respiratory failure associated with anatomical and functional stenotic lesions of the airway: a phase II clinical trial.
- Biallelic TEDC1 variants cause a new syndrome with severe growth impairment and endocrine complications.
- Surgical management of an ELBW neonatal PDA with congenital tracheal stenosis.Pediatrics international : official journal of the Japan Pediatric Society· 2025· PMID 40302028mais citado
- Computational fluid dynamics as an objective measure for assessing congenital tracheal stenosis: Proof-of-concept study.
- From Childhood to Adulthood: A Case of Congenital Tracheal Stenosis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:141127(Orphanet)
- OMIM OMIM:603569(OMIM)
- MONDO:0011340(MONDO)
- GARD:12008(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q28873218(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
