Primary Posterior Tracheopexy at Time of Esophageal Atresia Repair Significantly Reduces Respiratory Morbidity.

PURPOSE: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair. METHODS: Review of all newborn primary EA/TEF repairs (2016-2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. RESULTS: Among 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs -1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). CONCLUSION: Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. LEVEL OF EVIDENCE: Level III.

Open and Thoracoscopic Aortopexy for Airway Malacia in Children: 15 Year Single Centre Experience.

OBJECTIVES: The objective was to report and analyse the characteristics and results of open aortopexy and thoracoscopic aortopexy for the treatment of airway malacia in a paediatric population. METHODS: We report a retrospective consecutive case series of paediatric patients undergoing aortopexy for the treatment of airway malacia at a quaternary referral centre between December 2006 and January 2021. Outcome measures included days to extubation, continued need for non-invasive ventilation, further intervention in the form of tracheostomy and death. RESULTS: 169 patients underwent aortopexy: 147 had open procedures (135 via median/limited median sternotomy and 12 thoracotomy) and 22 thoracoscopic. Mean follow up was 8.46 yrs (range 1-20 yrs). Most common site of airway malacia was the trachea (n = 106, 62.7 %), and 48 (28.4 %) had additional involvement at the bronchi with tracheobronchomalacia (TBM). 15 (8.9 %) had bronchomalacia (BM) only. Incidence of bronchial disease was lower in the thoracoscopic than open group (13.6 % vs 40.82 %; p < 0.0001). Mean time to extubation was 1.45 days, 2.59 days, 5.23 days in tracheomalacia, TBM and BM groups, respectively (p = 0.0047). Mean time to extubation was 1.35 days, 2 days, 3.67 days, and 5 days in patients with external vascular compression, TOF/OA, primary airway malacia, and laryngeal reconstruction, respectively (p = 0.0002). There were 21 deaths across the cohort, and all were in the open group. 71.4 % (n = 15) had bronchial involvement of their airway malacia. CONCLUSIONS: Open and thoracoscopic aortopexy are effective treatments for airway malacia in children. We have identified that involvement of the bronchi is a risk factor for adverse outcomes, and the optimum treatment for this patient cohort is still debatable. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Retrospective Study.

The effect of bethanechol on tracheobronchomalacia in preterm infants with bronchopulmonary dysplasia: a retrospective cohort study.

OBJECTIVE: Bethanechol has demonstrated improvement in trachealis tone in animal models, but no trials have studied efficacy in infants. This study aimed to examine if bethanechol improves a standardized pulmonary severity score (PSS) in infants with severe bronchopulmonary dysplasia with a diagnosis of tracheobronchomalacia (TBM). STUDY DESIGN: This retrospective cohort study evaluated cases treated with bethanechol matched with controls who did not receive bethanechol. TBM was diagnosed by dynamic computography. Daily PSS was recorded for each infant from 40 to 55 weeks post-menstrual age. RESULTS: Cases' mean PSS change was 21% lower than the controls' mean PSS change pre- and post-bethanechol (95% CI -40%, -2%) by paired t-test (p = 0.03). Matched differences (controls' PSS - cases' PSS) demonstrated greater mean PSS difference post-bethanechol compared to pre-bethanechol 0.17, (95% CI 0.05, 0.29) by paired t-test (p = 0.009). CONCLUSION: Infants with TBM treated with bethanechol compared to those not treated had a lower PSS reflecting improved respiratory status.

Airway findings in trisomy 13 and trisomy 18: A 10-year retrospective review.

BACKGROUND AND OBJECTIVES: Trisomy 18 and trisomy 13 are the most common autosomal trisomies following trisomy 21, with overall incidence rising. Both diagnoses are characterized by multisystem involvement and were previously thought to be incompatible with life. New data suggest that prolonged survival is possible, and thus many families are opting for more aggressive medical interventions. This study aims to describe airway findings in trisomy 18 and trisomy 13, as these have not been comprehensively studied and can impact medical decision-making. We hypothesize that most children with trisomy 18 and trisomy 13 will have abnormal findings on airway endoscopy. METHODS: This a 10-year retrospective analysis of children with trisomy 13 or trisomy 18 who underwent endoscopic airway evaluation at a single center between 2011 and 2021. A total of 31 patients were evaluated. RESULTS: Thirty-one patients were included and underwent flexible bronchoscopy by a pediatric pulmonologist, often in conjunction with rigid bronchoscopy performed by pediatric otolaryngology. Findings were typically complimentary. All patients had at least one clinically significant finding on evaluation, and most patients had both upper and lower airway, as well as static and dynamic airway findings. The most common airway findings in children with trisomy 13 and 18 include tracheomalacia, bronchomalacia, laryngomalacia, hypopharyngeal collapse, glossoptosis, and bronchial compression. CONCLUSION: These findings can have significant implications for clinical care, and thus knowledge of trends has the potential to improve counseling on expected clinical course, presurgical planning, and informed consent before interventions.

Negative pressure suction test: An intraoperative airway maneuver to assess effectiveness of surgical correction of tracheobronchomalacia.

BACKGROUND: Surgical correction of tracheobronchomalacia (TBM) has evolved greatly over the past decade, with select pediatric institutions establishing dedicated surgery and anesthesia teams to navigate the complexities and challenges of surgical airway repairs. Although anesthetic techniques have evolved internally over many years to improve patient safety and outcomes, many of these methods remain undescribed in literature. TECHNIQUE: In this article, we describe the intraoperative negative pressure suction test. This simulates the negative pressure seen in awake and spontaneously breathing patients, including the higher pressures seen during coughing which induce airway collapse in patients with TBM. Also known as the Munoz maneuver in surgical literature, this test has been performed on over 300 patients since 2015. DISCUSSION: The negative pressure suction test allows for controlled intraoperative assessment of surgical airway repairs, replaces the need for risky intraoperative wake-up tests, increases the chances of a successful surgical repair, and improves anesthetic management for emergence and extubation. We provide a guide on how to perform the test and videos demonstrating its efficacy in intraoperative airway evaluation. CONCLUSIONS: As surgeries to repair TBM become more prevalent in other pediatric institutions, we believe that pediatric patients and anesthesia providers will benefit from the insights and methods described here.

Tracheobronchomalacia: an unusual cause of debilitating dyspnoea and its surgical management.

Tracheobronchomalacia (TBM) is a progressive weakening of the airways, leading to collapse and dyspnoea. TBM can be misdiagnosed when multiple chronic conditions accompany it. Tracheobronchoplasty (TBP) is indicated for severe symptomatic TBM, diagnosed by bronchoscopy and CT thorax. We report the case of a patient who underwent tracheal resection and reconstruction for continuing dyspnoea post argon therapy, TBP and a failure to tolerate extracorporeal membrane oxygenation-assisted Y-stent insertion. Relevant background history includes asthma, sleep apnoea, reflux, cardiomyopathy and a high body mass index. Bronchoscopy postreconstruction showed patent airways. Airway reconstruction was a viable management option for this patient's TBM. TBP is a treatment option for TBM. In this case, tracheal resection was required to sustain benefit. In addition, surveillance bronchoscopies will be carried out every year.

Single-center review on safety of biodegradable airway stenting in pediatric population.

BACKGROUND: Tracheobronchomalacia (TBM) and airway stenosis are recognized etiologies of airway obstruction among children. Their management is often challenging, requiring multiple interventions and prolonged respiratory support with associated long-term morbidity. Metallic or silicone stents have been used with mixed success and high complication rates. More recently biodegradable Ella stents (BES) provided an attractive interventional option. OBJECTIVES: We report our experience in the treatment of TBM and vascular airway compression using BES. We deliberately downsized them to minimize intraluminal granulation tissue formation. MATERIALS AND METHODS: Retrospective study over an 8-year period between November 2012 and December 2020 of pediatric patients with severe airway obstruction requiring airway stenting for extubation failure, malacic death spells, recurrent chest infections, or lung collapse. RESULTS: Thirty-three patients (5 tracheal and 28 bronchial diseases) required 55 BES during the study period. The smallest patient weighed 1.8 kg. Median age of patient at first stent implantation was 13.1 months (IQR 4.9-58.3). The majority of the bronchial stents were in the left main bronchus (93%), of which 57% for vascular compression. Repeat stents were used in 19 patients (57.7%), with a range of two to four times. We did not experience erosion, infection, or obstructive granuloma needing removal by forceps or lasering. Three stent grid occluded with secretions needing bronchoscopic lavage. Stent migration occurred in three patients. CONCLUSIONS: BES holds promise as a treatment option with low rate of adverse effects for a specific subset of pediatric patients with airway malacia or vascular compression. Further studies are warranted.

Dynamic computed tomography for evaluation of tracheobronchomalacia in premature infants with bronchopulmonary dysplasia.

INTRODUCTION: Dynamic computed tomography (dCT) gives real-time physiological information and objective descriptions of airway narrowing in tracheobronchomalacia (TBM). There is a paucity of literature in the evaluation of TBM by dCT in premature infants with bronchopulmonary dysplasia (BPD). The aim of this study is to describe the findings of dCT and resultant changes in management in premature infants with TBM. METHODS: A retrospective study of 70 infants was performed. Infants included were <32 weeks gestation without major anomalies. TBM was defined as ≥50% expiratory reduction in cross-sectional area with severity defined as mild (50%-75%), moderate (≥75%-90%), or severe (≥90%). RESULTS: Dynamic CT diagnosed malacia in 53% of infants. Tracheomalacia was identified in 49% of infants with severity as 76% mild, 18% moderate, and 6% severe. Bronchomalacia was identified in 43% of infants with varying severity (53% mild, 40% moderate, 7% severe). Resultant management changes included PEEP titration (44%), initiation of bethanechol (23%), planned tracheostomy (20%), extubation trial (13%), and inhaled ipratropium bromide (7%). CONCLUSION: Dynamic CT is a useful noninvasive diagnostic tool for airway evaluation of premature infants. Presence and severity of TBM can provide actionable information to guide more precise clinical decision making.

Thermoablative Techniques to Treat Excessive Central Airway Collapse.

Excessive central airway collapse (ECAC) is a condition characterized by the excessive narrowing of the trachea and mainstem bronchi during expiration, which can be caused by Tracheobronchomalacia (TBM) or Excessive Dynamic Airway Collapse (EDAC). The initial standard of care for central airway collapse is to address any underlying conditions such as asthma, COPD, and gastro-esophageal reflux. In severe cases, when medical treatment fails, a stent-trial is offered to determine if surgical correction is a viable option, and tracheobronchoplasty is suggested as a definitive treatment approach. Thermoablative bronchoscopic treatments, such as Argon plasma coagulation (APC) and laser techniques (potassium-titanyl-phosphate [KTP], holmium and yttrium aluminum pevroskyte [YAP]) are a promising alternative to traditional surgery. However, further research is needed to assess their safety and effectiveness in humans before being widely used.

How risky is it to remove an airway stent?

BACKGROUND: Airway stent removal has traditionally been associated with a high complication rate. Most studies on stent removal are over a decade old, prior to newer anti-cancer therapies, and by including non-contemporary and uncovered metal stents, may not reflect the current practices. We review our experience at Mount Sinai Hospital to report outcomes of stent removal with more contemporary practices. METHODS: A retrospective review was carried out of all the airway stent removals performed between 2018 to 2022, in adult patients with benign or malignant airway diseases. Stents inserted and removal for stent trials for tracheobronchomalacia were excluded from the final analysis. RESULTS: Forty-three airway stents removals in 25 patients were included. Twenty-five (58%) stents were removed in 10 patients with benign diseases, and 18 (42%) stents were removed in the remaining 15 patients with malignant diseases. Patients with benign disease were more likely to have their stent removed (OR 3.88). 63% of the stents removed were silicone. The most common reasons for stent removal were migration (n = 14, 31.1%) and treatment response (n = 13, 28.9%). Rigid bronchoscopy was used in 86% of cases. Ninety-eight percent of removals were accomplished in a single procedure. The median time to removal of stents was 32.5 days. Three complications were noted: hemorrhage (n = 1, 2.3%) and stridor (n = 2, 4.6%); one not directly related to the stent removal. CONCLUSIONS: Covered metal or silicone airway stents, in the era of contemporary stents, better cancer-directed therapies and surveillance bronchoscopies, can be removed safely with the use of rigid bronchoscopy.

External airway splint placement for severe pediatric tracheobronchomalacia.

OBJECTIVE: To present external airway splinting with bioabsorbable airway supportive devices (ASD) for severe, life-threatening cases of pediatric tracheomalacia (TM) or tracheobronchomalacia (TBM). METHODS: A retrospective cohort was performed for 5 pediatric patients with severe TM or TBM who underwent ASD placement. Devices were designed and 3D-printed from a bioabsorbable material, polycaprolactone (PCL). Pre-operative planning included 3-dimensional airway modeling of tracheal collapse and tracheal suture placement using nonlinear finite element (FE) methods. Pre-operative modeling revealed that triads along the ASD open edges and center were the most effective suture locations for optimizing airway patency. Pediatric cardiothoracic surgery and otolaryngology applied the ASDs by suspending the trachea to the ASD with synchronous bronchoscopy. Respiratory needs were trended for all cases. Data from pediatric patients with tracheostomy and diagnosis of TM or TBM, but without ASD, were included for discussion. RESULTS: Five patients (2 Females, 3 Males, ages 2-9 months at time of ASD) were included. Three patients were unable to wean from respiratory support after vascular ring division; all three weaned to room air post-ASD. Two patients received tracheostomies prior to ASD placement, but continued to experience apparent life-threatening events (ALTE) and required ventilation with supraphysiologic ventilator settings. One patient weaned respiratory support successfully after ASD placement. The last patient died post-ASD due to significant respiratory co-morbidity. CONCLUSION: ASD can significantly benefit patients with severe, unrelenting tracheomalacia or tracheobronchomalacia. Proper multidisciplinary case deliberation and selection are key to success with ASD. Pre-operative airway modeling allows proper suture placement to optimally address the underlying airway collapse.

A novel ex vivo tracheobronchomalacia model for airway stent testing and in vivo model refinement.

OBJECTIVES: We sought to develop an ex vivo trachea model capable of producing mild, moderate, and severe tracheobronchomalacia for optimizing airway stent design. We also aimed to determine the amount of cartilage resection required for achieving different tracheobronchomalacia grades that can be used in animal models. METHODS: We developed an ex vivo trachea test system that enabled video-based measurement of internal cross-sectional area as intratracheal pressure was cyclically varied for peak negative pressures of 20 to 80 cm H2O. Fresh ovine tracheas were induced with tracheobronchomalacia by single mid-anterior incision (n = 4), mid-anterior circumferential cartilage resection of 25% (n = 4), and 50% per cartilage ring (n = 4) along an approximately 3-cm length. Intact tracheas (n = 4) were used as control. All experimental tracheas were mounted and experimentally evaluated. In addition, helical stents of 2 different pitches (6 mm and 12 mm) and wire diameters (0.52 mm and 0.6 mm) were tested in tracheas with 25% (n = 3) and 50% (n = 3) circumferentially resected cartilage rings. The percentage collapse in tracheal cross-sectional area was calculated from the recorded video contours for each experiment. RESULTS: Ex vivo tracheas compromised by single incision and 25% and 50% circumferential cartilage resection produce tracheal collapse corresponding to clinical grades of mild, moderate, and severe tracheobronchomalacia, respectively. A single anterior cartilage incision produces saber-sheath type tracheobronchomalacia, whereas 25% and 50% circumferential cartilage resection produce circumferential tracheobronchomalacia. Stent testing enabled the selection of stent design parameters such that airway collapse associated with moderate and severe tracheobronchomalacia could be reduced to conform to, but not exceed, that of intact tracheas (12-mm pitch, 0.6-mm wire diameter). CONCLUSIONS: The ex vivo trachea model is a robust platform that enables systematic study and treatment of different grades and morphologies of airway collapse and tracheobronchomalacia. It is a novel tool for optimization of stent design before advancing to in vivo animal models.

Tracheobronchoplasty: Indications and Best Approaches.

Tracheobronchomalacia (TBM) is an increasingly recognized abnormality of the central airways in patients with respiratory symptoms. Severe TBM in symptomatic patients warrants screening dynamic CT of the chest and/or awake dynamic bronchoscopy. The goal of surgical repair is to restore the C-shaped configuration of the airway lumen and splint or secure the lax posterior membrane to the mesh to ameliorate symptoms. Robotic tracheobronchoplasty is safe and associated with improvements in pulmonary function and subjective improvement in quality of life.

Characteristics analysis of 157 cases of central airway stenosis due to tracheobronchial tuberculosis: A descriptive study.

Background: Tracheobronchial stenosis, particularly central airway stenosis, which frequently results in severe complications such as lung damage, occurs in patients with tracheobronchial tuberculosis (TBTB). Objectives: To analyze the clinical characteristics of patients with central airway stenosis due to tuberculosis (CASTB). Methods: Retrospective analysis was performed on the clinical features, radiological features, bronchoscopic features and treatment of 157 patients who were diagnosed with CASTB in two tertiary hospitals in Chongqing, China, from May 2020 to May 2022. Results: CASTB mostly occurs in young patients and females. Patients with CASTB exhibited different symptoms repeatedly during the disease, especially varying degrees of dyspnea, prompting many patients to undergo bronchoscopic intervention and even surgery. Patients with cicatricial strictures constituted the highest proportion of the TBTB subtype with CASTB and 35.7% of the patients with CASTB were found to have tracheobronchomalacia (TBM) under bronchoscopy. CASTB and TBM mainly involved the left main bronchus. Patients with lower levels of education had higher rates of TBM. Patients with TBM manifested shortness of breath more frequently than patients without TBM. Patients with TBTB who had undergone bronchoscopic interventions have a higher rate of TBM. Conclusions: Despite mostly adequate anti-tuberculosis chemotherapy, patients with TBTB can present with CASTB involving severe scarring stenosis, bronchial occlusion, tracheobronchomalacia and even destroyed lung.

Tracheopexy and aortopexy for circumflex aortic arch on a small infant.

Circumflex aortic arch is a rare vascular ring anomaly, which is often associated with tracheobronchomalacia. Aortic uncrossing is the definitive repair of this condition, which is a major high-risk operation; therefore, it is not suitable for small patients such as newborn infants or individuals who have severe comorbidities. We present a case of the circumflex aortic arch with tracheobronchomalacia in a 2.7-kg infant who underwent successful innovative combined surgery of the airway and the aorta.

Robotic Surgery for Tracheobronchomalacia.

Robotic tracheobronchoplasty (TBP) is a new surgical approach to treat tracheobronchomalacia (TBM), and is based on open TBP, which has undergone various technical iterations since the 1950s. The robotic approach to TBM may allow for more patients to undergo TBP, and in retrospective series has demonstrated equivalent outcomes compared to open TBP if not superior in terms of postoperative complications. Long-term data are eagerly awaited, and we describe our approach in this article.

Coordination of Care for Expiratory Central Airway Collapse: A Structured Process for a Multifaceted Disease.

Common respiratory symptoms, including dyspnea, cough, sputum production, and recurrent infections, frequently remain without a clear cause and may be the result of expiratory central airway collapse (ECAC). Establishing the diagnosis and appropriate treatment plan for patients with ECAC is challenging and benefits from a multidisciplinary approach. A coordinator role is crucial in this process to ensure optimal patient-centered outcomes. We describe the coordination of care in the process of diagnosing and treating ECAC. The coordinator leads the organization of the multiple services involved in the care of patients with ECAC, including pulmonary medicine, interventional pulmonology, radiology, and thoracic surgery, as well as hospital inpatient staff. From initial screening to evaluation and management with airway stents and corrective treatment with tracheobronchoplasty, the ECAC coordinator oversees the entire process of care for patients with ECAC.

Posterior tracheopexy for preterm infants with severe bronchopulmonary dysplasia and severe tracheobronchomalacia: A case series.

We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.

Paediatric tracheobronchomalacia: Incidence, patient characteristics, and predictors of surgical intervention.

OBJECTIVES: Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients. METHODS: We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis. RESULTS: 249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention. CONCLUSIONS: TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.