Surgical treatment for severe pediatric tracheobronchomalacia: the 20-year experience of a single center

Abstract Objective: In children with tracheobronchomalacia, surgical management should be reserved for the most severe cases and be specific to the type and location of tracheobronchomalacia. The goal of this study is to describe the presentation and outcomes of children with severe tracheobronchomalacia undergoing surgery. Methods: Retrospective case series of 20 children operated for severe tracheobronchomalacia at a tertiary hospital from 2003 to 2023. Data were collected on symptoms age at diagnosis, associated comorbidities, previous surgery, age at surgery, operative approach, time of follow-up, and outcome. Surgical success was defined as symptom improvement. Results: The most frequent symptoms of severe tracheobronchomalacia were stridor (50 %), cyanosis (50 %), and recurrent respiratory infections (45 %). All patients had one or more underlying conditions, most commonly esophageal atresia (40 %) and prematurity (35 %). Bronchoscopy were performed in all patients. Based on etiology, patients underwent the following procedures: anterior aortopexy (n = 15/75 %), posterior tracheopexy (n = 4/20 %), and/or posterior descending aortopexy (n = 4/20 %). Three patients underwent anterior aortopexy and posterior tracheopexy procedures. After a median follow-up of 12 months, 16 patients (80 %) had improvement in respiratory symptoms. Decannulation was achieved in three (37.5 %) out of eight patients with previous tracheotomy. The presence of dying spells at diagnosis was associated with surgical failure. Conclusions: Isolated or combined surgical procedures improved respiratory symptoms in 80 % of children with severe tracheobronchomalacia. The choice of procedure should be individualized and guided by etiology: anterior aortopexy for anterior compression, posterior tracheopexy for membranous intrusion, and posterior descending aortopexy for left bronchus obstruction.

Outcomes of surgical treatment of tracheobronchomalacia in children.

BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. MATERIALS AND METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data. RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. CONCLUSION: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

Lipoaspirate Injection in Relapsing Idiopathic Subglottic Stenosis: Preliminary Results.

OBJECTIVES: The management of idiopathic subglottic stenosis (iSGS) poses a clinical challenge due to high recurrence rates following both endoscopic and open approaches, often leading to tracheostomy. The activation of abnormal T-cells and cytokine pathways has been linked to iSGS pathogenesis. Autologous adipose tissue centrifugation yields lipoaspirate, offering optimal anti-inflammatory effects and biocompatibility widely utilized in various medical settings. This report presents the first 3 cases employing endoscopic dilation (ED) in combination with local lipoaspirate injection to address recurrent iSGS. METHODS: A prospective observational study was conducted, involving multidisciplinary evaluation by the Tracheal Team at the University of Modena. Patients meeting specific criteria were directed to undergo ED + lipoaspirate injection. RESULTS: Three patients fulfilled the inclusion criteria. The mean number of prior endoscopic procedures performed was 8. Endoscopic examination revealed 90% stenosis in patient A, 60% stenosis in patient B, and 60% stenosis in patient C. All patients presented inflammatory tissue or incipient granulations at the stenotic site, with an average time of 6 months between previous procedures. After 15 months, none of the patients required further procedures, and endoscopic examination revealed a significant reduction or disappearance of inflammatory tissue with a stable airway lumen. CONCLUSIONS: The observed results are encouraging in terms of reducing local inflammation and halting stenosis progression, especially in cases of short-term relapsing iSGS.

Fluoroscopically measured bronchial collapse in healthy dogs during cough exceeds 25%, and a cutoff of 60% bronchial collapse can be used to distinguish healthy from chronically coughing dogs.

Bronchomalacia, the weakening of bronchial cartilage, is a common cause of cough in dogs. The gold standard for diagnosis of bronchomalacia is bronchoscopy; however, fluoroscopy is often used as a preliminary diagnostic test due to its noninvasiveness. The normal amount of bronchial collapse in healthy dogs using fluoroscopy has not been previously established, with some authors citing <25%. The aims of this prospective and retrospective analytical observational study were to determine the normal amount of airway collapse in healthy animals, the amount of airway collapse in dogs with chronic cough and presumed bronchomalacia, and cut-off values of airway collapse during expiration and cough to distinguish between the two groups. Thoracic fluoroscopy was performed in right and left lateral recumbency during inspiration, expiration, and cough to evaluate the percentage collapse of the trachea and lobar bronchi in 45 healthy and 76 chronically coughing dogs. The bronchi of chronically coughing dogs exhibited a significantly greater amount of collapse during both expiration and cough compared with healthy dogs. Chronically coughing dogs exhibited up to 31.7 ± 26.6% collapse in expiration and 87.5 ± 23.7% collapse during cough, whereas healthy dogs exhibited 14.9 ± 6.1% collapse in expiration and 35.0 ± 13.7% collapse during cough. Based on ROC curves, the authors suggest using cut-off values of 30% during expiration and 60% during cough to distinguish between healthy dogs and those with presumed bronchomalacia. Findings suggest that though healthy dogs can exhibit a greater degree of airway collapse than previously thought, fluoroscopy can be used to distinguish between healthy and presumed bronchomalacic dogs.

Surgical treatment for severe pediatric tracheobronchomalacia: the 20-year experience of a single center.

OBJECTIVE: In children with tracheobronchomalacia, surgical management should be reserved for the most severe cases and be specific to the type and location of tracheobronchomalacia. The goal of this study is to describe the presentation and outcomes of children with severe tracheobronchomalacia undergoing surgery. METHODS: Retrospective case series of 20 children operated for severe tracheobronchomalacia at a tertiary hospital from 2003 to 2023. Data were collected on symptoms age at diagnosis, associated comorbidities, previous surgery, age at surgery, operative approach, time of follow-up, and outcome. Surgical success was defined as symptom improvement. RESULTS: The most frequent symptoms of severe tracheobronchomalacia were stridor (50 %), cyanosis (50 %), and recurrent respiratory infections (45 %). All patients had one or more underlying conditions, most commonly esophageal atresia (40 %) and prematurity (35 %). Bronchoscopy were performed in all patients. Based on etiology, patients underwent the following procedures: anterior aortopexy (n = 15/75 %), posterior tracheopexy (n = 4/20 %), and/or posterior descending aortopexy (n = 4/20 %). Three patients underwent anterior aortopexy and posterior tracheopexy procedures. After a median follow-up of 12 months, 16 patients (80 %) had improvement in respiratory symptoms. Decannulation was achieved in three (37.5 %) out of eight patients with previous tracheotomy. The presence of dying spells at diagnosis was associated with surgical failure. CONCLUSIONS: Isolated or combined surgical procedures improved respiratory symptoms in 80 % of children with severe tracheobronchomalacia. The choice of procedure should be individualized and guided by etiology: anterior aortopexy for anterior compression, posterior tracheopexy for membranous intrusion, and posterior descending aortopexy for left bronchus obstruction.

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.

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.

Bronchial collapse and bronchial stenting in 9 dogs.

BACKGROUND: Principal and lobar bronchial collapse is increasingly recognized as an isolated entity. OBJECTIVE: Retrospectively describe the procedure and outcomes of dogs undergoing bronchial stenting at a single referral hospital. ANIMALS: Nine client-owned dogs with variable degrees of collapse of the left principal bronchus (LPB), lobar bronchus 1 (LB1), and lobar bronchus 2 (LB2), and with clinically relevant signs of respiratory dysfunction. METHODS: Data were collected from patient records. All dogs underwent stenting of the LPB and LB2. Anatomic and functional impairment grades were assigned to each case before and 4 weeks after stenting. Data regarding response to stenting and complications were evaluated. RESULTS: Bronchial stenting was considered successful in all cases, with all dogs experiencing improved quality of life (QOL), and decreased functional impairment grade at 4 weeks post-stenting. Follow-up of >6 months was available for 6 dogs and of these, 5 were alive at 12 months, 3 were alive at 18 months, and 1 was alive at 24 months. Stent-related complications occurred in 4 dogs, and were resolvable in 3. Two dogs developed pneumothorax, 1 developed recurrent pneumonia, and 1 developed new-onset coughing. All dogs had mild and manageable coughing post-stenting. CONCLUSIONS AND CLINICAL IMPORTANCE: Stenting of the LBP and LB2 might be an effective option for dogs with advanced collapse of these bronchi and associated signs. Although all included dogs had resolution or improvement of clinical signs considered life-threatening or as affecting QOL, ongoing coughing is expected. Patient selection appears important with regard to achieving successful outcomes.

Quantifiable features of a tidal breathing phenotype in dogs with severe bronchomalacia diagnosed by bronchoscopy.

Dynamic lower airway obstruction is the primary component of canine bronchomalacia, but the ventilatory function remains underinvestigated. This prospective study analyzed tidal breathing characteristics in 28 dogs, comprising 14 with severe bronchomalacia diagnosed by bronchoscopy versus 14 without respiratory disease. Spirometry was conducted in all dogs. Bronchoscopy with bronchoalveolar lavage or brush under anesthesia was performed in 14 dogs with cough and expiratory effort. Severe bronchomalacia was defined by the severity of collapse and total number of bronchi affected. Ventilatory characteristics were compared between groups. Results revealed that dogs with severe bronchomalacia had lower minute volume (218 vs 338 mL/kg, p = .039) and greater expiratory-to-inspiratory time ratio (1.55 vs 1.35, p = .01) compared to control dogs. The tidal breathing pattern of dogs with bronchomalacia was different from that of normal dogs, and the pattern differed from the concave or flat expiratory curves typical of lower airway obstruction. Compared to control dogs, dogs with severe bronchomalacia had a significantly prolonged low-flow expiratory phase (p < .001) on the flow-time plot and a more exponential shape of the expiratory curve (p < .001) on the volume-time plot. Flow-time index ExpLF/Te (>0.14) and volume-time index Vt-AUCexp (≤31%) had a high ROC-AUC (1.00, 95% confidence interval 0.88 to 1.00) in predicting severe bronchomalacia. In conclusion, the tidal breathing pattern identified here indicates abnormal and complicated ventilatory mechanics in dogs with severe bronchomalacia. The role of this pulmonary functional phenotype should be investigated for disease progression and therapeutic monitoring in canine bronchomalacia.

Advantages of FDM and gamma irradiation to manufacture personalized medical devices for airway obstructions.

In the early childhood population, congenital airway conditions like bronchomalacia (BM) can pose a life-threatening threat. A breakthrough technology called additive manufacturing, or 3D printing, makes it feasible to create a biomedical device that aids in the treatment of airway obstruction. This article describes how a polycaprolactone (PCL) splint for the upper airways can be created using the fusion deposition technique (FDM) and sterilized using gamma radiation. It is presented as a simple, accessible, and cost-reduced alternative that complements other techniques using more expensive and sophisticated printing methods. Thermomechanical and morphological analysis proved that FDM and sterilizing by gamma irradiation are both appropriate methods for producing splints to treat life-threatening airway blockages. Additionally, the 3D-printed splints' effectiveness in treating a young patient with BM that was life-threatening was assessed by medical professionals. In this regard, the case report of a patient with 34 months of follow-up is presented. Splints manufactured by this affordable 3D printing method successfully surpass breathing arrest in life-threatening airway obstruction in pediatric patients. The success of this procedure represents a fundamental contribution to the treatment of the population in countries where access to expensive and complex technologies is not available.

Pre-treatment bronchoscopic evaluation in a case of relapsing polychondrits.

BACKGROUND: Relapsing polychondritis (RP) is a chronic and recurrent inflammatory disease of the cartilage tissues in the body. The cause of RP is unknown, and since it is a rare disease with symptoms that affect multiple organs, diagnosis is often delayed. CASE PRESENTATION: A 62-year-old woman with no smoking history visited our institution complaining of fever, cough, and dyspnoea. Chest CT showed a stenosis from the left main bronchus to the left lower lobe branch. Bronchoscopy visualised intense erythema and oedema at the left main bronchus, with airway narrowing. Biopsy of the ear revealed degenerative vitreous cartilage and fibrous connective tissue with a mild inflammatory cell infiltrate. She was subsequently diagnosed with RP and administered systemic corticosteroid therapy. Her symptoms improved rapidly, and post-treatment bronchoscopy revealed that although mild erythema of the airway epithelium remained, oedema markedly improved, and the airway stenosis was resolved. CONCLUSIONS: We report a case where pre-treatment bronchoscopy was able to visually confirm RP at the acute stage. Since RP is difficult to diagnose, severe airway narrowing can occur prior to diagnosis. Therefore, to determine the stage of the disease, it is helpful to perform bronchoscopic observation before treatment. However, bronchoscopic observation before treatment should be performed by experienced bronchoscopists due to the risk of airway obstruction.

Prospective evaluation of the efficacy of inhaled steroids administered via the AeroDawg spacing chamber in management of dogs with chronic cough.

BACKGROUND: Glucocorticoids are frequently required for management of cough because of inflammatory airway disease (IAD) and airway collapse (AWC). OBJECTIVES/HYPOTHESIS: To determine the efficacy and feasibility of inhaled administration of corticosteroids in controlling cough in dogs with noninfectious airway disease. ANIMALS: Thirty-six client-owned dogs. METHODS: Dogs were prospectively recruited for this placebo-controlled cross-over study. Inflammatory airway disease was diagnosed through bronchoalveolar lavage cytology. Airway collapse was diagnosed through bronchoscopy, or if dogs were unsuitable anesthetic candidates, by crackles on auscultation, radiographic changes in airway diameter, or fluoroscopy. Dogs were randomly assigned to receive placebo or fluticasone propionate for the first 2 weeks of the trial then crossed over to fluticasone. A quality of life (QOL) survey (best score 0, worst score 85) was completed at 0 and 6 weeks. A visual-analog cough survey was submitted at 0, 2, 4, and 6 weeks to assess cough, feasibility, and adverse effects of treatment. RESULTS: For 32 dogs, QOL score at study end (mean 11.3 ± 9.7) was significantly lower (P < .0001) compared to entry (mean 28.1 ± 14.1), with a median change of 69% in QOL score, indicating improved quality of life. Cough frequency, duration, and severity were significantly (P < .0001) decreased at study end. Feasibility of aerosolized delivery improved with continued use (P = .05) with only 1 dog unable to accept inhaled medication. CONCLUSION AND CLINICAL IMPORTANCE: This study supports the utility of fluticasone propionate by inhalation in management of cough in dogs with IAD and AWC.

Dynamic Volumetric Computed Tomography Angiography is an Effective Method to Evaluate Tracheomalacia in Children.

OBJECTIVE: Standard methods to evaluate tracheal pathology in children, including bronchoscopy, may require general anesthesia. Conventional dynamic proximal airway imaging in noncooperative children requires endotracheal intubation and/or medically induced apnea, which may affect airway mechanics and diagnostic performance. We describe a technique for unsedated dynamic volumetric computed tomography angiography (DV-CTA) of the proximal airway and surrounding vasculature in children and evaluate its performance compared to the reference-standard of rigid bronchoscopy. METHODS: Children who had undergone DV-CTA and bronchoscopy in one-year were retrospectively identified. Imaging studies were reviewed by an expert reader blinded to the bronchoscopy findings of primary or secondary tracheomalacia. Airway narrowing, if present, was characterized as static and/or dynamic, with tracheomalacia defined as >50% collapse of the tracheal cross-sectional area in exhalation. Pearson correlation was used for comparison. RESULTS: Over a 19-month period, we identified 32 children (median age 8 months, range 3-14 months) who had undergone DV-CTA and bronchoscopy within a 90-day period of each other. All studies were unsedated and free-breathing. The primary reasons for evaluation included noisy breathing, stridor, and screening for tracheomalacia. There was excellent agreement between DV-CTA and bronchoscopy for diagnosis of tracheomalacia (κ = 0.81, p < 0.001), which improved if children (n = 25) had the studies within 30 days of each other (κ = 0.91, p < 0.001). CTA provided incremental information on severity, and cause of secondary tracheomalacia. CONCLUSION: For most children, DV-CTA requires no sedation or respiratory manipulation and correlates strongly with bronchoscopy for the diagnosis of tracheomalacia. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:410-416, 2023.

Ventilator-Assisted Inspiratory and Expiratory Breath-Hold Thoracic Computed Tomographic Scans Can Detect Dynamic and Static Airway Collapse in Dogs with Limited Agreement with Tracheobronchoscopy.

Airway collapse (AC) in dogs includes a tracheal collapse, mainstem and lobar bronchial collapse, and bronchomalacia (i.e., segmental/subsegmental bronchial collapse). The clinical presentation of AC may overlap with non-collapsible airway disease (NCAD) or another non-lower airway respiratory disease (NLARD). This study determined whether paired inspiratory (I)/expiratory (E)-breath-hold computed tomography (I/E-BH CT) can detect a static and dynamic AC in dogs with spontaneous respiratory disease and it compared the CT-derived metrics of the AC to the tracheobronchoscopy metrics. The CT-acquired I and E diameter and cross-sectional area (CSA) for the trachea, mainstem and lobar bronchi in dogs with an AC (n = 16), NCAD (16), and NLARD (19) served for a dynamic percent of the airway narrowing (%AN) calculation. A scoring system assessed the bronchomalacia. The circularity was calculated for each airway. The results were compared to the tracheobronchoscopy collapse grading. In the dogs with an AC, the %AN was larger for the trachea, right mainstem bronchus and right middle lobar bronchus when they were compared to the dogs with NCAD and NLARD. Flattening was only identified for the trachea of the AC dogs. The agreement between the CT and tracheobronchoscopy scores was 20% from trachea to the lobar bronchi and 47% for the segmental/subsegmental bronchi. Paired I/E-BH CT can detect static and dynamic AC with limited agreement with the tracheobronchoscopy metrics. Independent scoring systems that are tailored to the clinical manifestations of functional impairments are needed.

Management of bronchomalacia in infants post-cardiac surgery using synchronized nasal DuoPAP: A novel technology.

Background: Tracheo-bronchomalacia (TBM) is the weakness in the structural integrity of the cartilaginous ring and arch. It may occur in isolation with prematurity or secondarily in association with various congenital anomalies. Bronchomalacia is more commonly associated with congenital heart diseases. The conventional treatment options include positive pressure ventilation with or without tracheostomy, surgical correction of external compression and airway stenting. Aim: To use "synchronized" nasal Dual positive airway pressure (DuoPAP), a non-invasive mode of ventilation as an alternative treatment option for bronchomalacia to avoid complications associated with conventional treatment modalities. Study Design: Prospective observational study conducted in Army Hospital Research and Referral from Jul 2019 to Dec 2020. Material and Methods: We diagnosed seven cases of TBM post-cardiac surgery at our institute, incidence of 4.2%. Four infants were diagnosed with left sided bronchomalacia, 2 were diagnosed with right sided bronchomalacia and one with tracheomalacia. Those infants were managed by "synchronized" nasal DuoPAP, a first in ventilation technology by Fabian Therapy Evolution ventilator (Acutronic, Switzerland). Results: All seven infants showed significant improvement with synchronized nasal DuoPAP both clinically as well as radiologically. None of the infant required tracheostomy and discharged to home successfully. Conclusion: The synchronized nasal DuoPAP is a low cost and effective treatment option for infants with TBM. It could be attributed to synchronization of the breaths leading to better tolerance and compliance in paediatric age group.

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.

Absent pulmonary valve with double-outlet left ventricle: a case report.

Absent pulmonary valve syndrome and double-outlet left ventricle are rare congenital anomalies, with, to the best of our knowledge, no cases reported to date. We present the treatment course in a patient with an absent pulmonary valve, double-outlet left ventricle, dextrocardia, hypoplastic right ventricle, valvular aortic stenosis, and bronchomalacia.

Synchronous airway lesions in children with severe, progressive and atypical laryngomalacia - Experience of a UK tertiary referral centre.

OBJECTIVES: Laryngomalacia is the commonest cause of stridor in the paediatric population. Whilst usually self-limiting, studies have suggested that the presence of synchronous airway lesions (SALs) might be associated with more severe clinical presentation and additional morbidity. However, evidence in the literature is scarce, contributing to controversy regarding the appropriate investigation and management of children with laryngomalacia. The aim of this study was to explore the prevalence, clinical significance and risk factors of SALs in children with severe, progressive and atypical laryngomalacia symptoms. METHODS: Retrospective analysis of the records of all paediatric patients diagnosed with laryngomalacia in a UK tertiary referral centre, over a 2-year period, was undertaken. Data on demographics, comorbidities, management and presence of SALs were collected. RESULTS: The study included 130 patients with laryngomalacia. Seventy-eight patients required investigation with microlaryngobronchoscopy (MLB), based on clinical presentation. SALs were identified in 22 patients (28.2%), 6 of which (7.7%) required surgical intervention. The commonest airway lesions included tracheomalacia, bronchomalacia and subglottic stenosis. A correlation between the presence of neurodevelopmental conditions in children and SALs was noted (p value < 0.05). The presence of SALs was not associated with sex, gestational age at birth or other medical comorbidities. CONCLUSION: The results of this study allow professionals to counsel families about the utility of MLB in children with severe, progressive and atypical laryngomalacia symptoms and inform management pathways for clinicians not working in airway centres. We support the clinical practice that progressive, atypical or severe laryngomalacia symptoms should prompt consideration of an MLB, to identify potential synchronous airway lesions. Furthermore, we propose that a higher index of suspicion and more thorough investigation might be indicated in children with neurodevelopmental disorders.

Resorbable airway splint, stents, and 3D reconstruction and printing of the airway in tracheobronchomalacia.

Tracheobronchomalacia (TBM) is the most common tracheobronchial obstruction. Most cases are mild to moderate; therefore, they do not need surgical treatment. Severe tracheomalacia, however, represents a diagnostic and therapeutic challenge since they are very heterogeneous. In the armamentarium of resources for the treatment of dynamic airway collapse, splints and stents are two underused strategies and yet, they may represent the best alternative in selected cases. Lately, computed tomography 3D reconstruction of the airway has been used for the design of virtual models that can be 3D-printed for the creation of novel devices to address training, simulation, and biotechnological implants for refractory and severe airway malformations. This manuscript examines the role of resorbable stents, splints, and the 3D reconstruction and printing of the pediatric airway in tracheobronchomalacia.

Advanced Therapies for Severe Tracheobronchomalacia: A Review of the Use of 3D-Printed, Patient-Specific, Externally Implanted, Bioresorbable Airway Splints.

Tracheobronchomalacia is a condition of dynamic collapse of the trachea and mainstem bronchi. The clinical significance of tracheobronchomalacia depends on its severity. Mild cases may be medically managed with limited symptomology, while severe cases require advanced therapies, lengthy hospital stays, and carry significant morbidity and mortality. Current therapies for severe tracheobronchomalacia include tracheostomy with prolonged mechanical ventilation, aortopexy, tracheobronchopexy, and intraluminal metallic, silicone, or bioresorbable stents. Three-dimensional (3D)-printed, patient-specific, bioresorbable airway splinting is a novel treatment option that is undergoing investigation in a cohort of critically ill children with severe tracheobronchomalacia. At the time of our last review of our data, 29 splints had been implanted in 15 children with intrathoracic tracheobronchomalacia. The median follow-up was 8.5 months. There were 12 long-term survivors, and all but one lived at home. This article discusses the details of our institution's development and use of 3D-printed, patient-specific, bioresorbable splints for treatment of severe tracheobronchomalacia in the pediatric population.