Raras
Buscar doenças, sintomas, genes...
Estenose traqueal, congênita
ORPHA:141127CID-10 · Q32.1CID-11 · LA73.0OMIM 603569DOENÇA RARA

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.

Mantido por Agente Raras·Colaborar como especialista →

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.

Publicações científicas
432 artigos
Último publicado: 2026 Feb
Medicamentos
1 registrados
EVEROLIMUS

Tem tratamento?

1 medicamento registrado
Ver detalhes, fases e interações →
EVEROLIMUS

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Canada
Início
Infancy
+ neonatal
🏥
SUS: Cobertura mínimaScore: 35%
Centros em: MG, PR, SC, RS, ES +10CID-10: Q32.1
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫁
Pulmão
8 sintomas
❤️
Coração
7 sintomas
🫃
Digestivo
2 sintomas
🫘
Rins
2 sintomas
👂
Ouvidos
1 sintomas
🦴
Ossos e articulações
1 sintomas

+ 15 sintomas em outras categorias

Características mais comuns

90%prev.
Choro fraco
Muito frequente (99-80%)
55%prev.
Sibilos
Frequente (79-30%)
55%prev.
Morfologia traqueobrônquica anormal
Frequente (79-30%)
55%prev.
Polidrâmnio
Frequente (79-30%)
55%prev.
Desconforto respiratório
Frequente (79-30%)
55%prev.
Escore de APGAR de 3 no 5º minuto
Frequente (79-30%)
36sintomas
Muito frequente (1)
Frequente (8)
Ocasional (23)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 36 características clínicas mais associadas, ordenadas por frequência.

Choro fracoWeak cry
Muito frequente (99-80%)90%
SibilosWheezing
Frequente (79-30%)55%
Morfologia traqueobrônquica anormalAbnormal tracheobronchial morphology
Frequente (79-30%)55%
PolidrâmnioPolyhydramnios
Frequente (79-30%)55%
Desconforto respiratórioRespiratory distress
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico432PubMed
Últimos 10 anos172publicações
Pico202231 papers
Linha do tempo
2026Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 1 medicamento · 0 ensaios
Carregando informações de tratamento...

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

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Infantil Albert Sabin

R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital de Apoio de Brasília (HAB)

AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital Universitário da UFJF

R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442

Atenção Especializada

Rota
Anomalias Congênitas

Hospital das Clínicas da UFMG

Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Julio Müller (HUJM)

R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092

Atenção Especializada

Rota
Anomalias Congênitas

Hospital Universitário João de Barros Barreto

R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Lauro Wanderley (HULW)

R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470

Atenção Especializada

Rota
Anomalias Congênitas

Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)

R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Pequeno Príncipe

R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805

Serviço de Referência

Rota
Anomalias CongênitasDeficiência Intelectual

Hospital Universitário Regional de Maringá (HUM)

Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108

Atenção Especializada

Rota
Anomalias Congênitas

Hospital de Clínicas da UFPR

R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Pedro Ernesto (HUPE-UERJ)

Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital São Lucas da PUCRS

Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário da UFSC (HU-UFSC)

R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da FMUSP

R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias Congênitas

Hospital de Clínicas da UNICAMP

R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

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

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

UNIFESP / Hospital São Paulo

R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo
Sobre os centros SUS: Estes centros são habilitados pelo Ministério da Saúde como Serviços de Referência em Doenças Raras ou Serviços de Atenção Especializada. O atendimento é pelo SUS, com encaminhamento da rede de atenção básica.

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.

🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
173 papers (10 anos)
#1

Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.

Cardiology in the young2026 Mar 05

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.

#2

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 Surgery2026 Jan 06

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.

#3

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.

Respiratory medicine2026 Jan

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.

#4

Biallelic TEDC1 variants cause a new syndrome with severe growth impairment and endocrine complications.

European journal of human genetics : EJHG2025 Jun

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.

#5

Surgical management of an ELBW neonatal PDA with congenital tracheal stenosis.

Pediatrics international : official journal of the Japan Pediatric Society2025

Publicações recentes

Ver todas no PubMed

📚 EuropePMC272 artigos no totalmostrando 171

2026

Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.

Cardiology in the young
2025

From Childhood to Adulthood: A Case of Congenital Tracheal Stenosis.

Cureus
2026

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

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.

Respiratory medicine
2025

Methodological considerations in the study of quality of life after slide tracheoplasty for congenital tracheal stenosis.

Journal of pediatric surgery
2025

Application of slide tracheoplasty in children with congenital tracheal stenosis.

Cardiology in the young
2025

Quality of Life and its Clinical Determinants After Slide Tracheoplasty for Congenital Tracheal Stenosis.

Journal of pediatric surgery
2025

Global research hotspots and trends in the field of surgical treatment of congenital tracheal stenosis in infants and children over the past 40 years: A bibliometric and visualization study.

Medicine
2025

The Inverted Steeple Sign, Tracheal Tug, and Biphasic Stridor: A Triad for Diagnosing Congenital Tracheal Stenosis.

Pediatric pulmonology
2025

Comparison of Postoperative Airflow Changes in Slide Tracheoplasty Across Surgical Techniques Using Computational Fluid Dynamics.

Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
2025

Surgical management of an ELBW neonatal PDA with congenital tracheal stenosis.

Pediatrics international : official journal of the Japan Pediatric Society
2025

Unexpected Difficult Intubation Reveals Congenital Tracheal Stenosis in an Adult During Anesthesia Induction for Gastroesophageal Junction Cancer Surgery.

Cureus
2025

Slide tracheoplasty without cardiopulmonary bypass for the treatment of long and complex congenital stenosis: case report and review of the literature.

Cirugia y cirujanos
2025

Biallelic TEDC1 variants cause a new syndrome with severe growth impairment and endocrine complications.

European journal of human genetics : EJHG
2024

Automated xeno-free chondrogenic differentiation from human embryonic stem cells: Enhancing efficiency and ensuring high-quality mass production.

Regenerative therapy
2025

Slide Tracheoplasty in Long Segment Tracheobronchial Stenosis.

The Annals of thoracic surgery
2024

Tracheal reconstructive surgery under ECMO for the treatment of congenital tracheal stenosis in the premature infant: case report.

Frontiers in pediatrics
2025

Congenital Tracheal Stenosis With Complete Cartilage Rings: Proposal of A Multidisciplinary and Tailored Surgical Approach.

Journal of pediatric surgery
2025

Enhancing epithelial regeneration with gelatin methacryloyl hydrogel loaded with extracellular vesicles derived from adipose mesenchymal stem cells for decellularized tracheal patching.

International journal of biological macromolecules
2025

Retrospective analysis of factors contributing to poor prognosis among 271 pediatric patients with tracheal stenosis treated with slide tracheoplasty.

The Journal of thoracic and cardiovascular surgery
2025

Airway Management With Congenital Tracheal Stenosis: Surgical and Anesthetic Consideration.

The Laryngoscope
2024

Achieving normal pulmonary function following tracheoplasty in infancy.

Interdisciplinary cardiovascular and thoracic surgery
2025

Tracheoplasty should be proactively considered in the surgical strategy for treating the ring-sling complex.

The Journal of thoracic and cardiovascular surgery
2024

The use of overlapping self-expandable covered stents in the management of long-segment tracheobronchomalacia: A case report.

Respirology case reports
2024

Slide tracheoplasty for congenital tracheal stenosis: A systematic review.

International journal of pediatric otorhinolaryngology
2024

Impact of preoperative respiratory distress on outcomes of slide tracheoplasty.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

Slide tracheoplasty for congenital tracheal stenosis with involvement of the carina and bronchi: a case report.

Einstein (Sao Paulo, Brazil)
2024

Aerodynamic evaluation of surgical design for the stenosis correction of airway.

Frontiers in cardiovascular medicine
2024

Congenital Tracheal Stenosis Patients Undergoing Modified Slide Tracheoplasty: Single-Centre Technique and Long-Term Morbidity and Mortality.

CJC pediatric and congenital heart disease
2024

Regeneration Process of an Autologous Tissue-Engineered Trachea (aTET) in a Rat Patch Tracheoplasty Model.

Bioengineering (Basel, Switzerland)
2024

Long- term outcomes of congenital tracheal stenosis after slide tracheoplasty.

Pediatric surgery international
2024

Case Report: ECMO-assisted tracheal reconstruction in a 30-week-gestation preterm infant with tracheal stenosis.

Frontiers in pediatrics
2024

Outcomes of not using tracheoplasty in asymptomatic tracheal stenosis found during open-heart surgery.

Interdisciplinary cardiovascular and thoracic surgery
2024

[Thoracoscopic tracheal resection in a child under extracorporeal membrane oxygenation].

Khirurgiia
2024

Familial congenital laryngotracheal stenosis: A systematic review.

International journal of pediatric otorhinolaryngology
2024

Composite Biosynthetic Graft for Repair of Long-Segment Tracheal Stenosis: A Pilot In Vivo and In Vitro Feasibility Study.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2024

TBX5 pathogenic variant in a patient with congenital heart defect and tracheal stenosis.

Congenital anomalies
2023

[Treatment strategy of congenital tracheal stenosis associated with non-vascular ring cardiac malformations].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2024

Congenital tracheal stenosis in Mowat-Wilson syndrome with nonsense mutation of ZEB2 gene.

Pediatrics and neonatology
2023

[A case of left pulmonary artery sling combined with congenital tracheal stenosis in an adult].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
2023

Partial median sternotomy approach for the repair of esophageal atresia.

Pediatrics international : official journal of the Japan Pediatric Society
2023

Treatment of long-segment congenital tracheal stenosis with congenital cardiovascular defects in infancy.

International journal of pediatric otorhinolaryngology
2023

Incidence of congenital tracheal stenosis in left pulmonary artery sling diagnosed by bronchoscopy.

Pediatric surgery international
2023

Preoperative Contrast Examinations Help Determine the Appropriate Cervical Approach for Congenital Gross Type C Esophageal Atresia: A Report of Two Cases.

The American journal of case reports
2023

Morphologic classification of tracheobronchial arborization in children with congenital tracheobronchial stenosis and the associated cardiovascular defects.

Frontiers in pediatrics
2023

Peripheral blood parameters for predicting PICU admission and mechanical ventilation in pediatric inpatients with human parainfluenza virus-induced pneumonia.

Journal of medical virology
2023

Use of Virtual Reality and Three-Dimensional Printing in the Surgical Planning of Slide Tracheoplasty.

World journal for pediatric &amp; congenital heart surgery
2023

Challenging Diagnosis of a Congenital Tracheal Malformation: Considerations From an Intensive Care Perspective.

Cureus
2023

Congenital nasal pyriform aperture stenosis; our experience of 34 cases.

International journal of pediatric otorhinolaryngology
2023

Tracheal reconstruction surgery for congenital tracheal stenosis.

Pediatric surgery international
2023

Missed bronchial web in a 4-year-old boy with foreign body aspiration: A case report.

Clinical case reports
2023

Sliding tracheoplasty of complete tracheal cartilage rings in children.

Journal of pediatric surgery
2022

A Review of Tracheal Bronchus in Universiti Kebangsaan Malaysia Medical Centre (UKMMC).

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2022

Slide Tracheoplasty: Complete Tracheal Rings and Beyond.

Otolaryngologic clinics of North America
2022

Tracheal necrosis due to tracheal tube cuff in congenital tracheal stenosis.

Pediatrics international : official journal of the Japan Pediatric Society
2022

The clinical application of flexible bronchoscopy in a neonatal intensive care unit.

Frontiers in pediatrics
2022

Simulated slide tracheoplasty for congenital tracheal stenosis using three-dimensional printed models.

Pediatric surgery international
2022

Computational fluid dynamics assessment of congenital tracheal stenosis.

Pediatric surgery international
2022

Surgical management of congenital tracheal stenosis associated with complex cardiovascular anomalies.

Pediatric surgery international
2022

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Evaluation and management of congenital tracheal stenosis.

International journal of pediatric otorhinolaryngology
2022

Successful surgical treatment of congenital tracheal stenosis combined with tracheal bronchus and left pulmonary artery sling: a 10-year single-institution experience.

Pediatric surgery international
2022

Airway performance in infants with congenital tracheal stenosis associated with unilateral pulmonary agenesis: effect of tracheal shape on energy flux.

Medical &amp; biological engineering &amp; computing
2023

Systematic Review of Slide Tracheoplasty Outcomes.

The Annals of otology, rhinology, and laryngology
2021

'Unventilable bronquiolitis' as symptom of congenital tracheal stenosis.

Archivos de bronconeumologia
2022

An Infant With Congenital Tracheal and Bronchial Stenosis Diagnosed by Chest Three-Dimensional Computed Tomography.

Cureus
2022

Respiratory viruses among pediatric inpatients with acute lower respiratory tract infections in Jinan, China, 2016-2019.

Journal of medical virology
2022

[Ring-sling complex association with pig bronchus: a pediatric case report].

Archivos argentinos de pediatria
2022

Outcomes of slide tracheoplasty for congenital tracheal stenosis in 80 children: A 22-year single-center experience.

Journal of pediatric surgery
2022

A case of successful slide tracheoplasty for long-segment congenital tracheal stenosis in a neonate with a congenital diaphragmatic hernia and Fallot's tetralogy.

Surgical case reports
2022

CT imaging for pulmonary artery sling with tracheal stenosis and hypoplastic right lung.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2022

Surgical outcomes of pulmonary artery sling and congenital tracheal stenosis with right lung anomaly.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2022

New insights in the surgical treatment of long-segment congenital tracheal stenosis.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2022

Commentary: Slide tracheoplasty for congenital tracheal stenosis: Sliding by the missing pieces.

The Journal of thoracic and cardiovascular surgery
2022

Tracheal Reconstruction for Congenital Tracheal Stenosis: A 950-Gram Neonate.

The Annals of thoracic surgery
2022

[Surgical tracheoplasty for children with congenital tracheal stenosis undergoing previous balloon dilatation or tracheal metal stent placement: a series of 9 cases].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2022

Surgical management of long-segment congenital tracheal stenosis with tracheobronchial malacia.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2022

Surgical management strategy of slide tracheoplasty for infants with congenital tracheal stenosis.

The Journal of thoracic and cardiovascular surgery
2022

Closed-PICU perioperative management of congenital tracheal stenosis.

Pediatrics international : official journal of the Japan Pediatric Society
2021

Surgical Treatment of Patients with Full Tracheal Rings: Our Experience.

Journal of laparoendoscopic &amp; advanced surgical techniques. Part A
2022

Congenital anomalies of the large intrathoracic airways.

Paediatric anaesthesia
2021

The Ring-and-Sling complex - Does it "Ring" true?

Indian journal of pathology &amp; microbiology
2022

Anomalous Origin of the Right Pulmonary Artery From the Aorta and Congenital Tracheal Stenosis: Staged Repair in a Neonate.

World journal for pediatric &amp; congenital heart surgery
2021

Combination therapy of continuous nebulization and high-flow nasal cannula for severe congenital tracheal stenosis.

Pediatric pulmonology
2021

Infectious complications after tracheoplasty for congenital tracheal stenosis: a retrospective comparative study.

Pediatric surgery international
2022

Slide Tracheoplasty for Congenital Tracheal Stenosis Repair: A Systematic Review and Meta-Analysis.

The Laryngoscope
2022

Incidence and risk factor of vocal cord paralysis following slide tracheoplasty for congenital tracheal stenosis: a retrospective observational study.

Cardiology in the young
2021

The Role of Lung Function Testing in Newborn Infants With Congenital Thoracic Arterial Anomalies.

Frontiers in pediatrics
2021

Congenital airway anomalies.

Seminars in pediatric surgery
2021

Successful airway management with combined use of a McGRATHTM MAC videolaryngoscope and fiberoptic bronchoscope in a patient with congenital tracheal stenosis diagnosed in adulthood.

JA clinical reports
2022

Risk Stratification of Slide Tracheoplasty for Pediatric Airway Stenosis.

The Annals of thoracic surgery
2021

Central airway obstruction treated with initial support by venovenous extracorporeal membrane oxygenation.

BMJ case reports
2021

Combination of slide tracheoplasty and side-to-side bronchoplasty for complex congenital tracheobronchial stenosis.

Interactive cardiovascular and thoracic surgery
2021

Improving community-based care for children with a rare condition: The example of long-segment congenital tracheal stenosis and perceptions of health professionals, parents and teachers.

International journal of pediatric otorhinolaryngology
2022

Lung Hypoplasia Associated With Ring-Sling Complex Is Usually Right-Sided.

The Annals of thoracic surgery
2021

Histological features of complete tracheal rings in congenital tracheal stenosis.

Pediatric surgery international
2021

Treatment of tracheal stenosis with extracorporeal membrane oxygenation support in infants and newborns.

Artificial organs
2020

Lethal severe congenital tracheal stenosis with tracheal ring complicating respiratory distress syndrome in an extremely premature infant: first reported case in Qatar with a literature review.

BMJ case reports
2020

Follow-Up Investigation of 41 Children After Metallic Airway Stent Implantation: An 8-Year Experience.

Frontiers in pediatrics
2020

Clinical Analysis of Congenital Deficient Tracheal Cartilage Rings: Six Case Reports and a Literature Review.

Frontiers in pediatrics
2020

A new dimension of success in the management of airway disease in children with neurological deficit.

International journal of pediatric otorhinolaryngology
2019

Isolated congenital tracheal stenosis: A rare and deadly condition.

Pediatric investigation
2020

Computational Evaluation of Surgical Design for Multisegmental Complex Congenital Tracheal Stenosis.

BioMed research international
2020

Difficult intubation and anesthetic management in an adult patient with undiagnosed congenital tracheal stenosis: a case report.

The Journal of international medical research
2020

Congenital tracheal stenosis & associated cardiac anomalies: operative management & techniques.

Journal of thoracic disease
2020

Staging of Surgical Procedures in Comorbid Congenital Tracheal Stenosis and Congenital Cardiovascular Disease.

The Annals of thoracic surgery
2020

Morphologic Analysis of Congenital Heart Disease With Anomalous Tracheobronchial Arborization.

The Annals of thoracic surgery
2019

Surgical indication for congenital tracheal stenosis complicated by pulmonary artery sling.

Journal of thoracic disease
2022

Tracheoplasty for Congenital Tracheal Stenosis with Bilateral Tracheal Bronchus.

Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
2019

Understanding the impact of slide tracheoplasty in congenital tracheal stenosis.

Translational pediatrics
2020

Clinical and structural aspects of tracheal stenosis and a novel embryological hypothesis of left pulmonary artery sling.

Pediatric pulmonology
2019

Effects of Different Modes of Mechanical Ventilation on Aerodynamics of the Patient-specific Airway: A Numerical Study.

Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference
2020

Outcome of congenital tracheal stenosis in children over two decades in a national cardiothoracic surgical unit.

Cardiology in the young
2019

[Congenital tracheal stenosis: from clinic to treatment.].

Revue medicale suisse
2019

Use of non-invasive ventilation in children with congenital tracheal stenosis.

International journal of pediatric otorhinolaryngology
2019

Non-operative management of congenital tracheal stenosis: criteria by computed tomography.

Pediatric surgery international
2019

Asymptomatic congenital tracheal stenosis.

Respirology case reports
2019

Spanish multicentre study on morbidity and pathogenicity of tracheal bronchus in children.

Pediatric pulmonology
2019

Excessive Tracheal Length in Patients With Congenital Tracheal Stenosis.

The Annals of thoracic surgery
2019

Long-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 Surgery
2019

A pilot study of a novel biodegradable magnesium alloy airway stent in a rabbit model.

International journal of pediatric otorhinolaryngology
2019

Tailored Management of Airway Abnormalities in Children with Congenital Single Functional Lung.

Journal of pediatric surgery
2019

Fluid dynamic assessment of tracheal flow in infants with congenital tracheal stenosis before and after surgery.

Medical &amp; biological engineering &amp; computing
2018

Congenital tracheal stenosis: what should we look at for successful tracheoplasty?

Translational pediatrics
2018

Pitfalls in the management of congenital tracheal stenosis: is conservative management feasible?

Pediatric surgery international
2018

Congenital tracheal malformations.

Pediatric surgery international
2019

Incidental Diagnosis of Congenital Tracheal Stenosis in Children With Congenital Heart Disease Presenting for Cardiac Surgery.

Journal of cardiothoracic and vascular anesthesia
2018

Enhanced chondrogenic differentiation of human mesenchymal stems cells on citric acid-modified chitosan hydrogel for tracheal cartilage regeneration applications.

RSC advances
2018

Revision thoracic slide tracheoplasty: Outcomes following unsuccessful tracheal reconstruction.

The Laryngoscope
2018

Anesthetic Management for Concomitant Correction of Congenital Cardiac Defects and Long "O" Ring Tracheal Stenosis: A Role for Heliox?: Report of 2 Cases.

A&amp;A practice
2018

Impact of 3D printing on the surgical management of tracheal stenosis associated to pulmonary sling: a case report.

Journal of thoracic disease
2018

Difficult intubation due to unknown congenital tracheal stenosis in the adult: a case report and literature review.

Journal of thoracic disease
2018

Swallowing outcomes in children after slide tracheoplasty.

International journal of pediatric otorhinolaryngology
2018

An approach to successful slide tracheoplasty in the low birth weight neonate with single lung.

International journal of pediatric otorhinolaryngology
2018

Pediatric Interventional Pulmonology.

Clinics in chest medicine
2018

Bronchial Mismatch as a Predictor of Respiratory Failure After Congenital Tracheal Stenosis Repair.

The Annals of thoracic surgery
2018

Clinical outcomes after tracheoplasty in patients with congenital tracheal stenosis in 1997-2014.

Journal of pediatric surgery
2017

To do or not to do? The management dilemma of congenital tracheal stenosis in the setting of the ring-sling complex.

Journal of thoracic disease
2017

Left pulmonary artery sling and congenital tracheal stenosis: to slide or not to slide?

Journal of thoracic disease
2017

Operative or non-operative treatment of congenital tracheal stenosis: is there something new?

Journal of thoracic disease
2018

Tracheal surgery in children: outcome of a 12-year survey.

Interactive cardiovascular and thoracic surgery
2018

Temporary 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 Surgery
2017

Slide tracheoplasty in 81 children: Improved outcomes with modified surgical technique and optimal surgical age.

Medicine
2017

[Surgical treatment of tracheal stenosis. A 11-year report in a pediatric hospital].

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
2019

Neonatal Tracheal and Intracardiac Repair in a High-Risk Premature Infant Requiring Preoperative ECMO Transport.

World journal for pediatric &amp; congenital heart surgery
2017

Management of congenital tracheal stenosis in the neonatal period.

Pediatric surgery international
2017

An Adolescent with Dyspnea and Cough. A Case of Congenital Tracheal Stenosis.

American journal of respiratory and critical care medicine
2017

Airway Problems in Neonates-A Review of the Current Investigation and Management Strategies.

Frontiers in pediatrics
2017

The role of adjunctive procedures in reducing postoperative tracheobronchial obstruction in single lung patients with congenital tracheal stenosis undergoing slide tracheoplasty.

Journal of pediatric surgery
2017

Pediatric airway surgery.

Pediatric surgery international
2016

Tracheal and bronchial stenoses and other obstructive conditions.

Journal of thoracic disease
2017

Slide tracheoplasty outcomes in children with congenital pulmonary malformations.

The Laryngoscope
2016

Biodegradable polydioxanone stent as a new treatment strategy for tracheal stenosis in a rabbit model.

Journal of pediatric surgery
2016

Advances in Surgical Treatment of Congenital Airway Disease.

Seminars in thoracic and cardiovascular surgery
2017

A case of pulmonary artery sling where onset Was induced by therapeutic hypothermia.

Pediatric pulmonology
2016

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis.

Pediatric surgery international
2016

Clinical equivalency of cardiopulmonary bypass and extracorporeal membrane oxygenation support for pediatric tracheal reconstruction.

Pediatric surgery international
2016

Congenital tracheobronchial stenosis.

Seminars in pediatric surgery
2016

Emergency management for congenital tracheal stenosis with endoluminal stenting in pediatric intensive care units.

Therapeutic advances in respiratory disease
2016

The association of congenital tracheobronchial stenosis and cardiovascular anomalies.

International journal of pediatric otorhinolaryngology
2016

Rapid Expansion of Human Epithelial Stem Cells Suitable for Airway Tissue Engineering.

American journal of respiratory and critical care medicine
2016

Bridging bronchus, type six, as a new rare case of a bronchial anomaly.

JA clinical reports
2016

Evolution of Surgical Approaches in the Management of Congenital Tracheal Stenosis: Single-Center Experience.

World journal for pediatric &amp; congenital heart surgery
2016

Abnormal cardiac axis as a prenatal marker of left pulmonary artery sling.

Pediatrics international : official journal of the Japan Pediatric Society
2015

Total Airway Reconstruction in the Neonate: Combined Mandibular Distraction and Slide Tracheoplasty for Multiple Level Airway Obstruction.

The Journal of craniofacial surgery
2015

[Anesthesia Management for Primary Cleft Lip Closure in an Infant with Congenital Total Length Complete Tracheal Rings].

Masui. The Japanese journal of anesthesiology
2016

Surgical 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 Surgery
2015

Management of Congenital Tracheal Stenosis.

Pediatrics
2014

[Advances in pediatric thoracic surgery during the past 50 years].

Nihon Geka Gakkai zasshi
2015

Management of tracheobronchial obstruction in infants using metallic stents: long-term outcome.

Pediatric surgery international
2014

Congenital tracheal stenosis: unanticipated and anticipated difficult airway management in a neonate.

A &amp; A case reports
2015

Modified end-to-end anastomosis for the treatment of congenital tracheal stenosis with a bridging bronchus.

The Annals of thoracic surgery
Ver todos os 272 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Estenose traqueal, congênita.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Estenose traqueal, congênita

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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.

  1. Right pulmonary agenesis with ventricular septal defect and partial anomalous left pulmonary artery sling: a case report.
    Cardiology in the young· 2026· PMID 41782239mais citado
  2. 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
  3. 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.
    Respiratory medicine· 2026· PMID 41412268mais citado
  4. Biallelic TEDC1 variants cause a new syndrome with severe growth impairment and endocrine complications.
    European journal of human genetics : EJHG· 2025· PMID 39979680mais citado
  5. Surgical management of an ELBW neonatal PDA with congenital tracheal stenosis.
    Pediatrics international : official journal of the Japan Pediatric Society· 2025· PMID 40302028mais citado
  6. Computational fluid dynamics as an objective measure for assessing congenital tracheal stenosis: Proof-of-concept study.
    JTCVS Open· 2026· PMID 41960111recente
  7. From Childhood to Adulthood: A Case of Congenital Tracheal Stenosis.
    Cureus· 2025· PMID 41458734recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:141127(Orphanet)
  2. OMIM OMIM:603569(OMIM)
  3. MONDO:0011340(MONDO)
  4. GARD:12008(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. 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

Estenose traqueal, congênita
Compêndio · Raras BR

Estenose traqueal, congênita

ORPHA:141127 · MONDO:0011340
Prevalência
Unknown
Herança
Not applicable
CID-10
Q32.1 · Outras malformações congênitas da traquéia
CID-11
Medicamentos
1 registrados
Início
Infancy, Neonatal
Prevalência
0.0 (Canada)
MedGen
UMLS
C0265767
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades