RESUMO
The trachea delivers inhaled air into the lungs for gas exchange. Anomalies in tracheal development can result in life-threatening malformations, such as tracheoesophageal fistula and tracheomalacia. Given the limitations of current therapeutic approaches, development of technologies for the reconstitution of a three-dimensional trachea from stem cells is urgently required. Recently, single-cell sequencing technologies and quantitative analyses from cell to tissue scale have been employed to decipher the cellular basis of tracheal morphogenesis. In this Review, recent advances in mammalian tracheal development and the generation of tracheal tissues from pluripotent stem cells are summarized.
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Pulmão/crescimento & desenvolvimento , Morfogênese/fisiologia , Traqueia/crescimento & desenvolvimento , Fístula Traqueoesofágica/patologia , Animais , Cartilagem/crescimento & desenvolvimento , Diferenciação Celular , Epitélio , Humanos , Mesoderma/crescimento & desenvolvimento , Camundongos , Morfogênese/genética , Sistema Respiratório , Traqueia/anormalidades , Traqueomalácia , TranscriptomaRESUMO
BACKGROUND: Biodegradable (BD) stents made from polydioxanone have been used only in human airways. These stents combine the advantages of classical tracheal stents, and fewer side effects are expected due to their biocompatibility and their time-limited presence in airways. However, new clinical consequences have arisen. Here, the authors share their experiences with BD stents for tracheal indications, focusing on their safety and efficacy. METHODS: This was a retrospective review of a collected database of adult patients who underwent implantation of biodegradable tracheal stents between September 2013 and December 2022 at the Department of Respiratory Medicine of the 1st Faculty of Medicine in Prague and Thomayer University Hospital. The indications included functionally significant nonmalignant tracheal stenosis and tracheomalacia. Self-expandable, biodegradable, polydioxanone tracheal stents manufactured by ELLA-CS Ltd. (Hradec Kralove, Czech Republic) were implanted during rigid bronchoscopy under general anaesthesia. All patients were followed up in the department and were provided with the necessary care. The main efficacy and safety parameters and relationships were analysed using descriptive statistics and Fisher´s exact, Wilcoxon and KruskalâWallis tests. RESULTS: A total of 65 stents were implanted in 47 adult patients. During the first two months after implantation, when adequate function was expected, the stent was found to be effective in 26 out of 39 patients who completed this period (66.7%). The clinical effectiveness reached 89.7%, as early restenoses were mostly mild and necessitated treatment in only 4 patients. The frequencies of significant mucostasis, migration and granulation tissue growth were 2.6%, 7.5% and 23.1%, respectively, during this period. Thirty-four participants completed the half-year follow-up period after the first or second stent insertion, and some were followed up beyond this period. Poor control of symptoms, the development of restenosis and the need for interventions were characteristic of this period as the stents degraded. Twenty-two patients who experienced remodelling or stabilization of the tracheal lumen achieved a stent-free state. Seven patients underwent subsequent surgical treatment. CONCLUSIONS: BD stents are safe and provide adequate tracheal support until they begin to degrade. The use of BD stents necessitates close monitoring of patients and accurate treatment of possible restenosis. TRIAL REGISTRATION: Based on project NT14146 - Biodegradable stents in the management of the large airways (2013-2015, MZ0/NT), registered on May 1, 2013, in the Research and Development and Innovation Information System of the Czech Republic and at ClinicalTrials.gov (reg. no. NCT02620319, December 2, 2015).
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Implantes Absorvíveis , Broncoscopia , Stents , Estenose Traqueal , Humanos , Pessoa de Meia-Idade , Masculino , Feminino , Estudos Retrospectivos , Adulto , Estenose Traqueal/cirurgia , Idoso , Polidioxanona , Traqueomalácia/cirurgia , República Tcheca , Resultado do Tratamento , Traqueia/cirurgia , Desenho de Prótese , Adulto JovemRESUMO
BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor. CASE PRESENTATION: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient's breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient's respiratory and cardiovascular function were stable. CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.
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Anestesia Geral , Broncoscopia , Sons Respiratórios , Humanos , Sons Respiratórios/etiologia , Masculino , Lactente , Anestesia Geral/métodos , Laringomalácia/complicações , Laringomalácia/diagnóstico , Laringomalácia/cirurgia , Traqueomalácia/complicações , Traqueomalácia/diagnóstico , Propofol , Ketamina/uso terapêuticoRESUMO
Despite improving the survival after repair of esophageal atresia (EA), the morbidity of EA repair remains high. Specifically, tracheomalacia (TM) is one of the most frequent complications of EA repair. Continuous positive airway pressure is generally applied for the treatment of TM. However, surgical intervention is required against an apparent life-threatening event or inability to perform extubation for a long period. According to our review, most cases of TM showed symptom improvement after aortopexy. The ratio of the trachea's lateral and anterior-posterior diameter at the brachiocephalic artery crossing the trachea, which reflects the compression of the trachea by the brachiocephalic artery, is a good indicator of aortopexy. Our finding suggests that most TM cases associated with EA may not be caused by tracheal fragility alone, but may involve blood vessel compression. Posterior tracheopexy (PT) is also an effective treatment for TM. Recently, open or thoracoscopic PT was able to be performed simultaneously with EA repair. In many cases, aortopexy or PT is a safe and effective surgical treatment for TM with EA. Other surgical procedures, such as external stenting, should be considered for patients with diffuse-type TM for whom aortopexy and PT appear relatively ineffective.
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Atresia Esofágica , Esofagoplastia , Traqueomalácia , Humanos , Lactente , Traqueomalácia/cirurgia , Traqueomalácia/complicações , Atresia Esofágica/cirurgia , Traqueia/cirurgia , Resultado do TratamentoRESUMO
INTRODUCTION: Subglottic and tracheal stenosis (SGTS) in adults is an acquired or idiopathic condition that can lead to dyspnea, and even life-threatening airway obstruction. Endoscopic techniques have advanced and largely eclipsed open surgery, with open surgery now reserved for refractory cases (Hseu et al., 2013; Feinstein et al., 2017). Currently, there is no accepted guideline for the endoscopic treatment of SGTS. Thus, the aim of the present study is to examine the impact of various clinical and pathological characteristics on outcomes to endoscopic treatment in a cohort of SGTS patients. DISCLOSURE: None of the authors have any financial or personal relationship that could cause a conflict of interest regarding this article. METHODS: Retrospective chart review was performed for 41 patients presenting with SGS without a tracheostomy over a 4-year-period (2018-2022), within a single tertiary care center. Quantitative outcomes including number of dilation procedures undergone and need for open procedures were examined. The qualitative variables included a history of pulmonary disease, prior tracheostomy/tracheal resection, presence of tracheomalacia, granulation tissue, excessive dynamic airway collapse (EDAC), and etiology of idiopathic subglottic stenosis. RESULTS: The presence of granulation tissue seen on tracheoscopy was associated with a higher number (4+) of dilation procedures (p = 0.01). A history of pulmonary disease (p = 0.037), the presence of tracheomalacia (p = 0.039), and the presence of granulation tissue (0.003) were all associated with a need for open procedures. CONCLUSION: Patients with the presence of granulation tissue, tracheomalacia, and a history of pulmonary disease were more associated with more severe disease requiring either a higher number of endoscopic procedures or need for open procedures.
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Laringoestenose , Pneumopatias , Estenose Traqueal , Traqueomalácia , Adulto , Humanos , Estenose Traqueal/etiologia , Estenose Traqueal/cirurgia , Traqueostomia/efeitos adversos , Estudos Retrospectivos , Traqueomalácia/complicações , Traqueomalácia/cirurgia , Resultado do Tratamento , Laringoestenose/cirurgia , Laringoestenose/complicações , Constrição Patológica , Pneumopatias/complicações , Pneumopatias/cirurgiaRESUMO
A tracheostomy is usually necessary for long-term mechanical ventilation or complicated weaning. Other indications include swallowing disorders with recurrent aspiration in neuromuscular disease and high-grade subglottic stenosis. The tracheostomy can be performed as a percutaneous dilatational tracheostomy or as a surgical tracheostomy. The complication rate is low, and intraoperative complications are differentiated from early and late postoperative complications. This article aims to present the indications, the techniques and complications of percutaneous dilatational and surgical tracheostomy, and highlights the long-term complications of tracheal stenosis and tracheomalacia.
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Complicações Pós-Operatórias , Estenose Traqueal , Traqueostomia , Humanos , Traqueostomia/métodos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Estenose Traqueal/cirurgia , Traqueomalácia/cirurgia , Traqueomalácia/etiologia , Dilatação/métodos , Complicações Intraoperatórias/etiologia , Complicações Intraoperatórias/prevenção & controle , Desmame do Respirador/métodos , Respiração Artificial/métodosRESUMO
Tracheal stenosis and tracheomalacia in patients with congenital scoliosis are serious and rare conditions caused by congenital dysplasia, postintubation injury, trauma, and tracheal tumor. Anesthesia of a child with tracheal stenosis is challenging for anesthesiologists. We describe an 8-year-old female patient developed severe tracheal stenosis and tracheomalacia after growing rod implantation for congenital scoliosis. Comprehensive assessment of preoperative pulmonary function and airway morphology, which can be neglected clinically, should be performed in congenital scoliosis patients.
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Escoliose , Estenose Traqueal , Traqueomalácia , Criança , Feminino , Humanos , Estenose Traqueal/cirurgia , Estenose Traqueal/complicações , Traqueomalácia/etiologia , Traqueomalácia/cirurgia , Escoliose/cirurgia , Escoliose/complicações , Traqueia/cirurgia , Traqueia/anormalidadesRESUMO
Operating on a huge and long-standing goiter is challenging to the surgeon and anesthetist because of the possibility of tracheomalacia and collapse of the tracheal rings after extubation. We report our innovation of tenting the trachea to the skin using sutures (passed through the strap and sternocleidomastoid muscles) to prevent post-thyroidectomy tracheomalacia.
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Bócio , Traqueomalácia , Humanos , Traqueia/cirurgia , Traqueostomia/efeitos adversos , Traqueomalácia/etiologia , Traqueomalácia/cirurgia , Tireoidectomia/efeitos adversos , Bócio/cirurgia , Bócio/complicaçõesRESUMO
BACKGROUND: Recurrent lower respiratory tract infections are among the most prevalent symptoms in secondary tracheomalacia due to mediastinal vascular anomalies (MVAs). It is not known whether this condition could result in persistent lower respiratory tract inflammation and subclinical infection. METHODS: A retrospective study was performed on records of children with tracheomalacia due to MVAs and recurrent respiratory infections who underwent computed tomography scan, bronchoscopy, and bronchoalveolar lavage (BAL) as part of their clinical evaluation. RESULTS: Thirty-one children were included in the study: 21 with aberrant innominate artery, four with right aortic arch, one with double aortic arch, and five with aberrant innominate artery associated with right aortic arch. Cytological evaluation of bronchoalveolar lavage fluid showed increased neutrophil percentages and normal lymphocyte and eosinophil proportions. Microorganism growth was detected in 13 BAL samples, with a bacterial load ≥104 colony-forming units/mL in eight (25.8%) of them. Most isolates were positive for Haemophilus influenzae. Bronchiectasis was detected in four children, all with BAL culture positive for H. influenzae. Four patients underwent MVA surgical correction and 27 conservative management, i.e., respiratory physiotherapy in all and high-dose amoxicillin/clavulanic acid (40 mg/kg/day) for 2-4 weeks in those with significant bacterial growth. There was an excellent outcome in most of them. CONCLUSIONS: Neutrophilic alveolitis is detectable in secondary tracheomalacia but is associated with a clinically significant bacterial load only in a quarter of the patients. Caution should be used regarding inappropriate antibiotic prescriptions to avoid the emergence of resistance, whilst airway clearance maneuvers and infection preventive measures should be promoted.
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Traqueomalácia , Criança , Humanos , Lactente , Traqueomalácia/complicações , Traqueomalácia/diagnóstico , Estudos Retrospectivos , Lavagem Broncoalveolar , Líquido da Lavagem Broncoalveolar , Inflamação , Broncoscopia , Sistema RespiratórioRESUMO
BACKGROUND: Esophageal atresia (EA) is often associated with tracheomalacia (TM). The severity of TM symptoms varies widely, with serious cases requiring prolonged respiratory support and surgical treatment. Although we performed thoracoscopic posterior tracheopexy (TPT) during primary EA repair to prevent or reduce the symptoms of TM, few studies have investigated the safety and effectiveness of TPT during primary EA repair. Therefore, this study aimed to evaluate the safety and efficacy of TPT in neonates. METHODS: We retrospectively reviewed the records of all patients diagnosed with TM who underwent primary thoracoscopic EA repair between 2013 and 2020 at the Nagoya University Hospital. Patients were divided into two groups: TPT (TPT group) and without TPT (control group). TPT has been performed in all patients with EA complicated by TM since 2020. We compared patient backgrounds, surgical outcomes, postoperative complications, and treatment efficacy. RESULTS: Of the 22 patients reviewed, eight were in the TPT group and 14 were in the control group. There were no statistically significant differences in the surgical outcomes between the groups (operation time: p = 0.31; blood loss: p = 0.83; time to extubation: p = 0.30; time to start enteral feeding: p = 0.19; time to start oral feeding: p = 0.43). Conversion to open thoracotomy was not performed in any case. The median operative time required for posterior tracheopexy was 10 (8-15) min. There were no statistically significant differences in postoperative complications between the groups (chylothorax: p = 0.36; leakage: p = 1.00; stricture: p = 0.53). The respiratory dependence rate 30 days postoperative (2 [25%] vs. 11 [79%], p = 0.03) and the ratio of the lateral and anterior-posterior diameter of the trachea (LAR) were significantly lower in the TPT group (1.83 [1.66-2.78] vs. 3.59 [1.80-7.70], p = 0.01). CONCLUSIONS: TPT during primary EA repair for treatment of TM significantly lowered respiratory dependence rate at 30 days postoperative without increasing the risk of postoperative complications. This study suggested that TPT could improve TM associated with EA.
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Atresia Esofágica , Traqueomalácia , Estudos de Coortes , Atresia Esofágica/complicações , Atresia Esofágica/cirurgia , Humanos , Recém-Nascido , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Toracoscopia , Traqueomalácia/complicações , Traqueomalácia/diagnóstico , Traqueomalácia/cirurgia , Resultado do TratamentoRESUMO
PURPOSE: Tracheomalacia (TM) is a frequent complication after esophageal atresia (EA) repair. This study aimed to review patients who underwent aortopexy for TM after EA repair and to compare their imaging features. METHODS: The patients who underwent thoracoscopic EA repair and contrast-enhanced computed tomography (CECT) at our hospital between 2013 and 2020 were retrospectively reviewed. The ratio of the lateral and anterior-posterior diameter of the trachea (LAR) where the brachiocephalic artery (BCA) crosses the trachea was defined. The LAR of the patients who underwent CECT for asymptomatic pulmonary disease was set as a normal reference. The Z-score of each LAR was calculated and compared between the patients that did or did not undergo aortopexy. RESULTS: A total of 51 patients represented the controls, 5 patients underwent aortopexy, and 12 patients were discharged without surgery. The mean LARs in the patients who underwent aortopexy, did not undergo aortopexy, and controls were 3.54, 1.54, and 1.15, respectively. The mean Z-score of the aortopexy group was 21.2. After successful aortopexy, each patient's LAR decreased to < 1.5. CONCLUSION: Aortopexy was preferred if the trachea was compressed by the BCA. The LAR is a useful indicator for predicting the therapeutic effect of aortopexy.
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Atresia Esofágica , Doenças da Traqueia , Traqueomalácia , Artérias , Atresia Esofágica/complicações , Atresia Esofágica/diagnóstico por imagem , Atresia Esofágica/cirurgia , Humanos , Lactente , Estudos Retrospectivos , Toracoscopia , Doenças da Traqueia/diagnóstico por imagem , Doenças da Traqueia/etiologia , Doenças da Traqueia/cirurgia , Traqueomalácia/diagnóstico por imagem , Traqueomalácia/cirurgiaRESUMO
BACKGROUND: H type tracheoesophageal fistula (H-TEF) is a rare congenital anomaly. Management may be complicated by late diagnosis and variation(s) in the therapeutic strategy. A systematic review of published studies explores the utility of diagnostic studies, operations and postoperative complications. METHODS: Medline and PubMed database(s) were searched for ALL studies reporting H-TEF during 1997-2020. Using PRISMA methodology, manuscripts were screened for eligibility and reporting. RESULTS: Forty-seven eligible studies were analysed. Primary diagnosis varied widely with surgeons performing oesophagography and trachea-bronchoscopy. Preoperative localisation techniques included fluoroscopy, guidewire placement and catheterisation. A cervical approach (209 of 272 cases), as well as thoracotomy, thoracoscopy and endoscopic fistula ligation, were all described. Morbidity included fistula recurrence (1.7%), leak (2%), tracheomalacia (3.4%) and respiratory sequelae (1%). The major adverse complication in all studies was vocal cord palsy secondary to laryngeal nerve injury (18.5%) yet strikingly few centres routinely reported undertaking vocal cord screening pre or postoperatively. CONCLUSION: This study shows that paediatric surgeons record low volume activity with H type tracheoesophageal fistula. Variation(s) in clinical practice are widely evident. Laryngeal nerve injury and its subsequent management warrant special consideration. Care pathways may offset attendant morbidity and define 'best practice.'
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Traumatismos do Nervo Laríngeo Recorrente/etiologia , Fístula Traqueoesofágica/diagnóstico , Fístula Traqueoesofágica/terapia , Broncoscopia , Feminino , Humanos , Recém-Nascido , Masculino , Complicações Pós-Operatórias/etiologia , Período Pós-Operatório , Estudos Retrospectivos , Toracoscopia , Toracotomia/efeitos adversos , Traqueia/cirurgia , TraqueomaláciaRESUMO
Tracheomalacia (TM) is characterized by tracheal collapse due to an intrinsic anomaly resulting in a lack of rigidity of the cartilaginous rings and/or the posterior membrane during expiration, coughing or crying. It may also be secondary to external compression or acquired during endobronchial diseases. TM is commonly associated with other syndromes or airway abnormalities. Tracheomalacia can be localized or diffused and if the main bronchi are involved, the term of tracheobronchomalacia (TBM) is used. The most common symptoms include expiratory stridor, barking cough and recurrent respiratory tract infections. If tracheal weakness is severe, Acute Life Threating Events (ALTE) or Brief Resolved Unexplained Event (BRUE) can occur. While mild forms usually do not require any treatment, severe TBM may require medical and/or surgical management. Amongst several possible treatments, including tracheostomy, noninvasive ventilation and airway stenting, the pexy surgical approach (posterior, anterior tracheopexy or aortopexy) is currently the favoured option.
La trachéomalacie (TM) est caractérisée par un collapsus trachéal plus ou moins important durant l'expiration, lors des efforts de toux ou des pleurs. Elle peut être due à une anomalie intrinsèque, par manque de rigidité des anneaux cartilagineux et/ou de la membrane postérieure. Elle peut aussi avoir une origine extrinsèque, soit secondaire à une compression externe, soit acquise dans le cadre de pathologies endo-bronchiques. Elle peut enfin être associée à certains syndromes ou malformations des voies respiratoires. La TM peut être localisée ou généralisée, et si les bronches principales sont atteintes, on parlera de trachéobronchomalacie (TBM). Les symptômes les plus courants sont : un stridor expiratoire, une toux aboyante, et des infections respiratoires récurrentes. Dans les cas les plus sévères, des événements menaçant la vie de l'enfant (Acute Life-Threatening Event «ALTE¼ ou Brief Resolved Unexplained Event «BRUE¼) peuvent survenir. Alors que les formes légères ne requièrent généralement pas de traitement, la TBM sévère peut nécessiter une prise en charge médicale et/ou chirurgicale. Parmi les divers choix thérapeutiques, incluant notamment la trachéostomie, la ventilation non invasive et les stents trachéaux, l'approche chirurgicale par pexie (aortopexie, trachéopexie postérieure ou antérieure) est actuellement l'option favorite.
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Traqueobroncomalácia , Traqueomalácia , Brônquios , Criança , Tosse , Humanos , Traqueia , Traqueobroncomalácia/diagnóstico , Traqueobroncomalácia/terapia , Traqueomalácia/diagnóstico , Traqueomalácia/terapiaRESUMO
Hunter Syndrome is a genetic disease characterized by deficiency of Iduronate-2-Sulfatase enzyme activity, resulting in accumulation of glycoaminoglycans in various organs including the central airways. We report a case of severe tracheomalacia and airway stenosis at Hospital Sultanah Aminah, Johor Bahru, Malaysia requiring mechanical ventilation in a middle aged gentleman who was previously undiagnosed of mucopolysaccharidosis. The patient underwent emergency tracheostomy for failed intubation, when he presented with shortness of breath and acute respiratory failure. A contrast-enhanced computed tomography of the neck and thorax revealed that the trachea distal to the tracheostomy tube had collapsed with narrowed right and left main bronchus. These findings were confirmed via direct visualization of the airway through a flexible bronchoscopy. Eventually, a tracheal stenting were performed to maintain the airway patency and assist in weaning off from mechanical ventilation. Further investigations to identify the aetiology of the central airway stenosis revealed elevated urinary glycoaminoglycans and the absence of iduronate-2-Sulfatase activity tested on dried blood spots, thus confirming the diagnosis of Hunter Syndrome. Managing mucopolysacharidosis with central airway obstruction requires multidisciplinary team effort in handling the difficult airway, anaesthesiology risk, potential comorbidities and providing genetic counselling.
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Obstrução das Vias Respiratórias , Mucopolissacaridose II , Traqueomalácia , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Broncoscopia , Constrição Patológica , Humanos , Masculino , Pessoa de Meia-Idade , Mucopolissacaridose II/complicações , Mucopolissacaridose II/diagnóstico , Traqueomalácia/diagnóstico por imagem , Traqueomalácia/etiologia , TraqueostomiaRESUMO
We report one-stage radical surgical treatment of a 54-year-old patient with extensive cicatricial posttracheostomy tracheal stenosis complicated by tracheomalacia of anterior tracheal wall and previous numerous unsuccessful endoscopic attempts of tracheal recanalization. A new method of prevention of tracheal anastomosis failure was applied.
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Anastomose Cirúrgica/métodos , Traqueia/cirurgia , Estenose Traqueal/cirurgia , Traqueomalácia/cirurgia , Traqueostomia/efeitos adversos , Humanos , Pessoa de Meia-Idade , Traqueia/lesões , Estenose Traqueal/etiologia , Traqueomalácia/etiologiaRESUMO
Tracheomalacia and tracheobronchomalacia may be primary abnormalities of the large airways or associated with a wide variety of congenital and acquired conditions. The evidence on diagnosis, classification and management is scant. There is no universally accepted classification of severity. Clinical presentation includes early-onset stridor or fixed wheeze, recurrent infections, brassy cough and even near-death attacks, depending on the site and severity of the lesion. Diagnosis is usually made by flexible bronchoscopy in a free-breathing child but may also be shown by other dynamic imaging techniques such as low-contrast volume bronchography, computed tomography or magnetic resonance imaging. Lung function testing can provide supportive evidence but is not diagnostic. Management may be medical or surgical, depending on the nature and severity of the lesions, but the evidence base for any therapy is limited. While medical options that include bronchodilators, anti-muscarinic agents, mucolytics and antibiotics (as well as treatment of comorbidities and associated conditions) are used, there is currently little evidence for benefit. Chest physiotherapy is commonly prescribed, but the evidence base is poor. When symptoms are severe, surgical options include aortopexy or posterior tracheopexy, tracheal resection of short affected segments, internal stents and external airway splinting. If respiratory support is needed, continuous positive airway pressure is the most commonly used modality either via a face mask or tracheostomy. Parents of children with tracheobronchomalacia report diagnostic delays and anxieties about how to manage their child's condition, and want more information. There is a need for more research to establish an evidence base for malacia. This European Respiratory Society statement provides a review of the current literature to inform future study.
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Broncomalácia/diagnóstico por imagem , Broncomalácia/terapia , Pneumologia/normas , Traqueomalácia/diagnóstico por imagem , Traqueomalácia/terapia , Broncoscopia , Criança , Europa (Continente) , Humanos , Imageamento por Ressonância Magnética , Tomografia Computadorizada Multidetectores , Modalidades de Fisioterapia , Pneumologia/organização & administração , Testes de Função Respiratória , Sons Respiratórios , Sociedades MédicasRESUMO
BACKGROUND: Neonatal dynamic tracheal collapse (tracheomalacia, TM) is a common and serious comorbidity in infants, particularly those with chronic lung disease of prematurity (bronchopulmonary dysplasia, BPD) or congenital airway or lung-related conditions such as congenital diaphragmatic hernia (CDH), but the underlying pathology, impact on clinical outcomes, and response to therapy are not well understood. There is a pressing clinical need for an accurate, objective, and safe assessment of neonatal TM. PURPOSE: To use retrospectively respiratory-gated ultrashort echo-time (UTE) MRI to noninvasively analyze moving tracheal anatomy for regional, quantitative evaluation of dynamic airway collapse in quiet-breathing, nonsedated neonates. STUDY TYPE: Prospective. POPULATION/SUBJECTS: Twenty-seven neonatal subjects with varying respiratory morbidities (control, BPD, CDH, abnormal polysomnogram). FIELD STRENGTH/SEQUENCE: High-resolution 3D radial UTE MRI (0.7 mm isotropic) on 1.5T scanner sited in the neonatal intensive care unit. ASSESSMENT: Images were retrospectively respiratory-gated using the motion-modulated time-course of the k-space center. Tracheal surfaces were generated from segmentations of end-expiration/inspiration images and analyzed geometrically along the tracheal length to calculate percent-change in luminal cross-sectional area (A % ) and ratio of minor-to-major diameters at end-expiration (r D,exp ). Geometric results were compared to clinically available bronchoscopic findings (n = 14). STATISTICAL TESTS: Two-sample t-test. RESULTS: Maximum A % significantly identified subjects with/without a bronchoscopic TM diagnosis (with: 46.9 ± 10.0%; without: 27.0 ± 5.8%; P < 0.001), as did minimum r D,exp (with: 0.346 ± 0.146; without: 0.671 ± 0.218; P = 0.008). Subjects with severe BPD exhibited a far larger range of minimum r D,exp than subjects with mild/moderate BPD or controls (0.631 ± 0.222, 0.782 ± 0.075, and 0.776 ± 0.030, respectively), while minimum r D,exp was reduced in CDH subjects (0.331 ± 0.171) compared with controls (P < 0.001). DATA CONCLUSION: Respiratory-gated UTE MRI can quantitatively and safely evaluate neonatal dynamic tracheal collapse, as validated with the clinical standard of bronchoscopy, without requiring invasive procedures, anesthesia, or ionizing radiation. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2019;49:659-667.
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Displasia Broncopulmonar/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Traqueomalácia/diagnóstico por imagem , Broncoscopia/métodos , Comorbidade , Feminino , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Humanos , Processamento de Imagem Assistida por Computador , Recém-Nascido , Terapia Intensiva Neonatal , Masculino , Estudos Prospectivos , Respiração , Resultado do TratamentoRESUMO
OBJECTIVE: To evaluate outcomes of patients with esophageal atresia (EA) on systematic treatment with proton pump inhibitors (PPI) since the neonatal period and to determine factors associated with successful discontinuation of PPI. STUDY DESIGN: Longitudinal cohort study with prospective data collection of 73 EA patients, over 11 years systematically treated with PPI. Outcome and predictive factors for discontinuation of PPI treatment were evaluated at study end in February 2017. The incidence of anastomotic strictures was compared with a historical cohort of 134 EA patients followed in the same institution between 1990 and 2005 before the era of systematic PPI treatment. RESULTS: PPI treatment was discontinued definitively in 48% of patients during follow-up. Prematurity, longer initial hospitalization, moderate-to-severe tracheomalacia, anastomotic leak and anastomotic stricture had a significant negative association with PPI discontinuation on univariate analysis (Pâ<â0.05). On adjusted multivariable Cox regression analysis, moderate-to-severe tracheomalacia and anastomotic leak were negatively associated with discontinuation of PPI treatment (hazard ratio 0.26 [95% CI 0.12-0.59]; Pâ=â0.001 and hazard ratio 0.38 [95% CI 0.16-0.93]; Pâ=â0.03, respectively). There was no significant difference in the incidence of anastomotic strictures in the present cohort compared with the historical cohort (44% vs 39%); (Pâ>â0.05). CONCLUSIONS: PPI treatment does not prevent the formation of anastomotic strictures and appears to be over-prescribed in children with airway symptoms because of tracheomalacia. This suggests that PPI treatment could be prescribed more selectively. Close monitoring and long-term follow-up, however, of these vulnerable patients in specialized multidisciplinary clinics is imperative.
Assuntos
Atresia Esofágica/cirurgia , Esôfago/cirurgia , Refluxo Gastroesofágico/tratamento farmacológico , Lansoprazol/uso terapêutico , Inibidores da Bomba de Prótons/uso terapêutico , Fístula Traqueoesofágica/cirurgia , Anastomose Cirúrgica/efeitos adversos , Fístula Anastomótica/etiologia , Criança , Pré-Escolar , Constrição Patológica/etiologia , Atresia Esofágica/complicações , Monitoramento do pH Esofágico , Feminino , Refluxo Gastroesofágico/etiologia , Humanos , Lactente , Estudos Longitudinais , Masculino , Período Pós-Operatório , Fístula Traqueoesofágica/complicações , Traqueomalácia/complicações , Resultado do TratamentoRESUMO
OBJECTIVE: The aim of this study was to determine the average effective radiation dose and feasibility of ultralow dose dynamic expiratory computed tomography (CT) for evaluation of tracheomalacia (ULD) and to evaluate factors that impact image quality. METHODS: This is a retrospective study of 64 consecutive patients from September to October 2016 for the evaluation of tracheomalacia. All studies were performed with routine inspiration chest CT followed by ULD z(kilovoltage peak (kVp) 80, 100, or 120 and fixed milliamperage 10) or typical dose CT (TD) (kVp 100 or 120 with automated milliamperage) dynamic expiration CT. Image quality was considered diagnostic if the trachea area could be accurately measured for tracheomalacia assessment, and diagnostic studies were graded fair, good, or excellent. Scan length, image quality, and effective radiation dose were compared for ULD versus TD and ULD at 100 kVp versus ULD at 80 kVp. For ULD studies, patient factors were compared across image quality. RESULTS: The ULD had a mean effective radiation dose of 0.08 mSv, with all studies of diagnostic image quality. The ULD showed 95% reduction in effective radiation dose (P < 0.001), 14% significant reduction in scan length (P = 0.029), and qualitatively decreased image quality compared w2 ith TD (P < 0.001). The ULD at 100 kVp had significantly better image quality compared with ULD at 80 kVp (P = 0.041) with higher effective radiation dose (0.09 vs 0.05 mSv) (P < 0.001). Body mass index significantly impacted image quality for all ULD studies but not for ULD at 80 or 100 kVp. CONCLUSION: For evaluation of tracheomalacia, ULD showed low effective radiation dose less than 0.1 mSv and maintained diagnostic image quality.
Assuntos
Tomografia Computadorizada por Raios X/métodos , Traqueomalácia/diagnóstico por imagem , Idoso , Estudos de Viabilidade , Feminino , Humanos , Masculino , Doses de Radiação , Reprodutibilidade dos Testes , Estudos Retrospectivos , Razão Sinal-Ruído , Traqueia/diagnóstico por imagemRESUMO
BACKGROUND: Intrathoracic goitres (ITG) often present with compressive symptoms and require specialised care by experienced surgical teams. Most ITG can be accessed by a transcervical approach (TCA) and only between 1 and 15% will require an extracervical approach (ECA). Many controversies exist regarding the clinical presentation, evaluation, selection of cases for ECA, surgical technique and outcomes. This paper reviews the recent literature on the management, outcomes and evidence-based treatment strategies of ITG. METHODS: We conducted a review of the literature on the evaluation, management and outcomes of surgery for ITGs. RESULTS: The incidence of cancer in the ITGs ranges between 4 and 20%. Multiplanar CT scanning offers the best preoperative evaluation and aids to determine the approach. Most ITG can be accessed by TCA and ECA are only needed in maximum 15% of cases. In experienced hands, the outcome of these surgeries is comparable to thyroid surgery for non-ITG. CONCLUSIONS: Surgery for ITG is challenging. The experienced surgeon however, with few exceptions can address ITG via TCA, with outcomes comparable to those of uncomplicated thyroid surgery.