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Tracheomalacia causes considerable morbidity in children, and the best treatment options remain debated. This paper presents a case series of seven Danish children who underwent surgical interventions, such as tracheopexy and aortopexy, demonstrating favourable clinical outcomes, notably with early intervention. We discuss the indications, timing and potential benefits of surgery for tracheomalacia in reducing respiratory symptoms caused by tracheal collapse. Our case series highlights the potential of surgical options in managing tracheomalacia, emphasising the need for standardised protocols, multidisciplinary and international collaboration, and further research to optimise treatment strategies and outcomes. [ABSTRACT FROM AUTHOR]

Vascular Rings (VRs) are congenital malformations of the aortic arch that can compress the trachea and esophagus producing symptoms. Approximately, 5%-10% of patients experience persistent symptoms postrepair and 9% require reintervention. There are various approaches to repair—simple ligation and division of the ring or more complex procedures. Our objective was to describe outcomes for VR repair using a "simple" surgical approach. We identified patients who underwent VR repair from 2012 to 2022 at our institution. Clinical and surgical data, demographics, intraoperative, and postoperative outcomes were extracted from their electronic medical records. Outcomes were analyzed and regression analysis was used to identify risk factors for residual symptoms after repair. Ninety three patients with VR repair were included. Prevalence of symptoms were reduced following surgery (80% preoperative versus 13% postoperative, P 0.03). Tracheomalacia also decreased from 16% to 7% postrepair. No deaths were reported in our cohort and 2% of our patients required reintervention. Multivariable regression analysis showed that preoperative tracheomalacia was associated with having persistent symptoms after VR repair (odds ratio 6.2, 95% CI 1.02-37.6). Our institutional experience using a simple surgical approach showed a significant decrease in symptoms, a very low reintervention rate and no mortality. Preoperative tracheomalacia can be a risk factor for persistent symptoms postrepair. We believe a simple surgical approach for children with complete VR repair can be effective in relieving symptoms. [ABSTRACT FROM AUTHOR]

Introduction Posterior tracheopexy (PT) directly addresses the posterior trachealis membrane intrusion in severe tracheomalacia. During PT, the esophagus is mobilized and membranous trachea is sutured to the prevertebral fascia. Although dysphagia has been reported as a possible complication of PT, in the literature there are no data investigating postoperative esophageal anatomy and digestive symptoms. Our aim was to study clinical and radiological consequences of PT on esophagus. Methods Patients with symptomatic tracheobronchomalacia scheduled for PT between May 2019 and November 2022 underwent pre- and postoperative esophagogram. For each patient, we analyzed radiological images and measured esophageal deviation providing new radiological parameters. Results All 12 patients underwent thoracoscopic PT (n = 3) or robot-assisted thoracoscopic PT (n = 9). For all patients, the postoperative esophagogram showed a right dislocation of the thoracic esophagus (median postoperative deviation = 27.5 mm). We report an esophageal perforation at postoperative day 7 in a patient affected by esophageal atresia, who underwent several surgical procedures before. A stent was placed and esophagus healed. Another patient with severe right dislocation referred transient dysphagia to solids, which resolved gradually in the first postoperative year. All the other patients did not present any esophageal symptoms. Conclusion For the first time, we demonstrate the right dislocation of the esophagus after PT and we propose an objective method to measure it. In most patients, PT is a procedure not affecting esophageal function, but dysphagia can occur if dislocation is important. Esophagus mobilization during PT should be cautious, especially in patients who underwent previous thoracic procedures. [ABSTRACT FROM AUTHOR]

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.

Introduction: Lower airway malacia (LAM) is characterized by a reduction in the cross-sectional luminal area during quiet respiration. There is no gold standard diagnostic test; however, flexible fiberoptic bronchoscopy (FFB) is most frequently utilized. The exact prevalence and incidence of LAM are unknown. This study aimed to determine the prevalence rates of pediatric patients diagnosed with LAM, offer a detailed understanding of their demographic and clinical characteristics, and investigate distinctions between two specific types of LAM, namely, tracheomalacia (TM) and bronchomalacia (BM). Materials and Methods: Patients younger than 18 years diagnosed with LAM using FFB were included in this retrospective case series. Demographic and clinical characteristics and comorbid disorders were compared between patients with isolated BM and those with isolated TM or tracheobronchomalacia (TM/TBM). Results: Among 390 patients who underwent FFB, 65 (16.6%) were diagnosed with LAM, 16 (24.6%) with TM, and 56 (86.2%) with BM. The median age at diagnosis was 15 months. Among them, 59 (90.8%) had other comorbidities; gastrointestinal (GI) disorders were the most common (38.5%). The most common indications for bronchoscopy were recurrent/prolonged lower respiratory tract infections (LRTI) or wheezing (43.1%), while the most frequently observed respiratory physical examination finding was stridor (35.4%). Patients with TM/TBM had significantly higher frequencies of premature births, stridor, retraction, and GI disorders. Conclusion: Patients with stridor without typical laryngomalacia features or recurrent or prolonged LRTI should undergo prompt evaluation for LAM. The potential coexistence of GI disorders such as gastroesophageal reflux disease and swallowing dysfunction should also be considered. [ABSTRACT FROM AUTHOR]

Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term. A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up. 121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent. Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately. • What is currently known about this topic? Short-term airway outcomes from large series and long-term outcomes for patients referred for treatment at otolaryngology/aerodigestive centres • What new information is contained in this article? This study describes long-term airway outcomes for all patients seen in our centre with TOF/OA not just those who have required specialist airway intervention. [ABSTRACT FROM AUTHOR]

Historically, children afflicted with long gap esophageal atresia (LGEA) had few options, either esophageal replacement or a life of gastrostomy feeds. In 1997, John Foker from Minnesota revolutionized the treatment of LGEA. His new procedure focused on "traction-induced growth" when the proximal and distal esophageal segments were too far apart for primary repair. Foker's approach involved placement of pledgeted sutures on both esophageal pouches connected to an externalized traction system which could be serially tightened, allowing for tension-induced esophageal growth and a delayed primary repair. Despite its potential, the Foker process was received with criticism and disbelief, and to this day, controversy remains regarding its mechanism of action - esophageal growth versus stretch. Nonetheless, early adopters such as Rusty Jennings of Boston embraced Foker's central principle that " one's own esophagus is best " and was instrumental to the implementation and rise in popularity of the Foker process. The downstream effects of this emphasis on esophageal preservation would uncover the need for a focused yet multidisciplinary approach to the many challenges that EA children face beyond "just the esophagus", leading to the first Esophageal and Airway Treatment Center for children. Consequently, the development of new techniques for the multidimensional care of the LGEA child evolved such as the posterior tracheopexy for associated tracheomalacia, the supercharged jejunal interposition, as well as minimally invasive internalized esophageal traction systems. We recognize the work of Foker and Jennings as key catalysts of an era of esophageal preservation and multidisciplinary care of children with EA. [ABSTRACT FROM AUTHOR]

Tracheobronchomalacia (TBM) occurs due to the weakening of cartilaginous part of the trachea, resulting in compromised airway function and leading to symptoms such as dyspnea, cough, and inability to clear secretions. Bronchiolitis obliterans syndrome (BOS) is the most prevalent late noninfectious pulmonary complication in patients who underwent allogeneic haematopoietic stem cell transplantation (HSCT). Therefore, patients experiencing progressive dyspnea and chronic cough after allogenic HSCT, with new obstructive pattern on pulmonary function test, are typically diagnosed with post‐transplant BOS. However, it is important to note that TBM can also manifest as an obstructive defect pattern on pulmonary function test. Tracheomalacia has been reported as a rare complication of allogenic stem cell transplantation. We present two patients who developed TBM following allogeneic HSCT and were initially treated for post‐transplant BOS but did not experience symptom improvement. However, after treatment with continuous positive airway pressure, their symptom subsided. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the role infant pulmonary function tests (Tidal Breathing Flow Volume Loops, TBFVL) in children with airway anomalies and to correlate the TBFVL so obtained with bronchoscopy findings. Methods: In this prospective cohort study, we enrolled children aged 0–2 years with airway anomalies and performed TBFVL and bronchoscopy. The primary outcome measure was graphic pattern of TBFVL in laryngomalacia. Secondary outcome measures were types of TBFVL results in various airway anomalies and controls. Results: Out of 53 children enrolled, 28 (52.3%) had laryngomalacia. Pattern 3 (fluttering of inspiratory limb) was commonest TBFVL pattern in laryngomalacia. Among TBFVL parameters, the ratio of inspiratory time to expiratory time (Ti/Te) and tPTEF/tE was significantly high in children with isolated laryngomalacia compared to controls. At six months of follow-up, TBFVL pattern 1 (normal) became the commonest pattern. Conclusion: A particular type of airway anomaly may have a characteristic graphic pattern in TBFVL and TBFVL pattern may indicate improvement in airway anomalies in follow-up. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: The Koolen-de Vries syndrome (KdVS) is a relatively new rare disease caused by micro-deletion of 17q21.31 which was first reported by Koolen in 2006. Typical phenotypes for KdVS include hypotonia, developmental delay, moderate intellectual disability, and characteristic facial dysmorphism. Up to now, there was only one case report about anesthesia management of patient diagnosed KdVS. It was a 2-year-old girl who experienced an MRI exam under anesthesia. Case presentation: We described a 21-month-old boy who planned to undergo an orchidopexy under general anesthesia diagnosed with KdVS. He had an intellectual disability, characteristic facial dysmorphism, tracheo/laryngomalacia, patent foramen ovale, and cryptorchidism related to KdVS. Due to the complex condition especially the presence of tracheo/laryngomalacia, we took some special measures, including reducing the amount of long-acting opioid, keeping the spontaneous breath, performing a caudal block, and applying the laryngeal mask. But the laryngeal mask was changed to an endotracheal tube because it failed to provide adequate ventilation. The boy experienced mild laryngeal spasm and hypoxia after extubation, but lateral position and etomidate eased his breathing problem and re-intubation was avoided. It is indicated that anesthesia management for patients with orphan disease is a real challenge for all anesthesia providers. Conclusions: The Koolen-de Vries syndrome is a relatively new orphan disease involving multiple systems. Keeping spontaneous breath, evaluating airway potency to anesthetics, applying endotracheal tube, and post-extubation lateral or prone position may be helpful for airway management for patient with hypotonia and tracheo/laryngomalacia. KdVS patient needs prolonged post-anesthesia monitoring and/or medication for airway complications. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Rationale: Neonates with respiratory issues are frequently treated with aerosolized medications to manage lung disease or facilitate airway clearance. Dynamic tracheal collapse (tracheomalacia [TM]) is a common comorbidity in these patients, but it is unknown whether the presence of TM alters the delivery of aerosolized drugs. Objectives: To quantify the effect of neonatal TM on the delivery of aerosolized drugs. Methods: Fourteen infant subjects with respiratory abnormalities were recruited; seven with TM and seven without TM. Respiratory-gated 3D ultrashort echo time magnetic resonance imaging (MRI) was acquired covering the central airway and lungs. For each subject, a computational fluid dynamics simulation modeled the airflow and particle transport in the central airway based on patient-specific airway anatomy, motion, and airflow rates derived from MRI. Results: Less aerosolized drug reached the distal airways in subjects with TM than in subjects without TM: of the total drug delivered, less particle mass passed through the main bronchi in subjects with TM compared with subjects without TM (33% vs. 47%, p = 0.013). In subjects with TM, more inhaled particles were deposited on the surface of the airway (48% vs. 25%, p = 0.003). This effect becomes greater with larger particle sizes and is significant for particles with a diameter >2 μm (2–5 μm, p ≤ 0.025 and 5–15 μm, p = 0.004). Conclusions: Neonatal patients with TM receive less aerosolized drug delivered to the lungs than subjects without TM. Currently, infants with lung disease and TM may not be receiving adequate and/or expected medication. Particles >2 μm in diameter are likely to deposit on the surface of the airway due to anatomical constrictions such as reduced tracheal and glottal cross-sectional area in neonates with TM. This problem could be alleviated by delivering smaller aerosolized particles. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. IV. Retrospective Study. • Aortopexy is an effective treatment for large airway malacia in a paediatric population. • Bronchial involvement is a risk factor for prolonged intubation, tracheostomy and mortality. • Thoracoscopic approach has excellent outcomes for select patients and has a role for those with favourable anatomy. • Optimum treatment for bronchial involvement requires further studies. [ABSTRACT FROM AUTHOR]

Down Syndrome (DS) is the most common chromosomal abnormality compatible with life. The life of patients suffering from DS can be strongly impacted by Recurrent Respiratory tract Infections (RRIs), leading to an increased rate of hospitalisation, a higher need for intensive care and fatality. With a literature review, we summarise here the main etiological factors for RRI in this category of patients, particularly focusing on airway malformations such as tracheomalacia, tracheal bronchus and bronchomalacia, comorbidities associated with the syndrome, like congenital heart diseases, dysphagia, gastroesophageal reflux, musculoskeletal involvement and obesity, and immunologic impairments, involving both innate and adaptive immunity. For these patients, a multidisciplinary approach is imperative as well as some preventive strategies, in particular vaccinations in accordance with their national schedule for immunization. [ABSTRACT FROM AUTHOR]

Abstract Tracheobronchomalacia (TBM) occurs due to the weakening of cartilaginous part of the trachea, resulting in compromised airway function and leading to symptoms such as dyspnea, cough, and inability to clear secretions. Bronchiolitis obliterans syndrome (BOS) is the most prevalent late noninfectious pulmonary complication in patients who underwent allogeneic haematopoietic stem cell transplantation (HSCT). Therefore, patients experiencing progressive dyspnea and chronic cough after allogenic HSCT, with new obstructive pattern on pulmonary function test, are typically diagnosed with post‐transplant BOS. However, it is important to note that TBM can also manifest as an obstructive defect pattern on pulmonary function test. Tracheomalacia has been reported as a rare complication of allogenic stem cell transplantation. We present two patients who developed TBM following allogeneic HSCT and were initially treated for post‐transplant BOS but did not experience symptom improvement. However, after treatment with continuous positive airway pressure, their symptom subsided.

Background: This paper reports the innovative use of a modified nasopharyngeal airway device as a temporary stent in patients with laryngotracheal stenosis. It also discusses the technique of endoscopic stent placement, and our experience in terms of the indications and suitability. Method: The nasopharyngeal airway device was modified to use as an airway stent by trimming it to the desired length. Next, the stent was inserted endoscopically and anchored using a novel approach. Results: The surgery was performed successfully without complications. The patients had full use of their voice while the stent was in situ. No significant granulation tissue was observed. Conclusion: This paper demonstrates the feasibility of using a nasopharyngeal airway device as a temporary stent to prevent restenosis in cases where the patients have a strong demand for phonation. The modified nasopharyngeal airway device is potentially very promising, but cases must be selected carefully to avoid compromising efficacy and safety. [ABSTRACT FROM AUTHOR]

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. 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. 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). Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. Level III. [ABSTRACT FROM AUTHOR]

A congenital complete tracheal ring is an infrequent cause of tracheal stenosis. It is usually diagnosed in infants and young children, and adults diagnosed with complete tracheal rings are even rarer entities. There is limited literature regarding the adult complete tracheal ring. Down syndrome patients have a wide array of documented anatomic variations especially airway abnormalities such as laryngomalacia, tracheomalacia, tracheal bronchus and bronchomalacia and rare to have complete tracheal ring. Hence, we described a Down syndrome adult who remained asymptomatic until the recent onset of severe respiratory illness, which requires endotracheal intubation, leading to an unexpectedly difficult airway. A differential diagnosis of a complete tracheal ring should be considered when attending such cases of difficult intubation. [ABSTRACT FROM AUTHOR]

To show the incidence of airway complications in ICU. Endotracheal intubation is an essential skill performed by multiple medical specialists to secure a patient's airway as well as provide oxygenation and ventilation through the oral route or nose. The goal of endotracheal intubation in the emergency setting is to secure the patient's airway and obtain first-pass success. There are many indications for endotracheal intubation, including poor respiratory drive, questionable airway patency, hypoxia, and Hypercapnia. These indications are assessed by evaluating the patient's mental status, conditions that may compromise the airway, level of consciousness, respiratory rate, respiratory acidosis, and level of oxygenation. In the setting of trauma, a Glasgow Coma Scale of 8 or less is generally an indication for intubation. There are many different complications of intubation as hoarseness of voice, dental injuries, arytenoid dislocation, laryngeal stenosis, tracheal stenosis and tracheomalacia.. 150 patients who were sat in the ICU that developed certain complications. 86 patients (57.3%) were sitting in the ICU develoed certain complications. Liver diseases were the main cause of ICU admission 34 (22.7%) patients then shock 32 (21.3%) patients. Blockage of endotracheal tube was the main ICU complications 18 (12%) patients then sinusitis 16 (10.7%) patients. Endotracheal intubation is a lifesaving procedure and its complications are significant problems in ICUs. A successful procedure of intubation avoids complications. Skilled endotracheal intubation in the ICU decreases the complications. [ABSTRACT FROM AUTHOR]

Tracheal agenesis (TA) is a rare congenital anomaly with an incidence of 1 per 50,000 newborns. It appears at birth with severe respiratory distress, cyanosis, and inaudible crying. Prompt esophageal intubation and long-term management of the esophageal airway are essential to overcome this catastrophic condition. In the long-term management, external stenting of the esophageal airway has been reported as promising to support the fragile esophageal wall; this technique was taken from the surgery for tracheomalacia. We experienced a case of an infant with tracheal agenesis whose respiratory status was stabilized after external esophageal stenting. The stenting was performed based on a lesson learned in the extensive experience in the surgical treatment for tracheomalacia, and the surgical techniques for successful stenting are herein described. [ABSTRACT FROM AUTHOR]

Abstract Background Members of anesthesia teams are involved in the management of COVID-19 infection cases at many stages particularly during acute airway interventions. Here, we describe a series of airway procedures to manage complex post-COVID-19 tracheomalacia in a high-risk patient. Case presentation A 67-year-old female with a medical history of diabetes mellitus, hypertension, and morbid obesity and recent COVID-19 infection requiring prolonged intubation presented to our hospital in severe respiratory distress. She was found to have long-segment tracheomalacia requiring multiple airway surgical procedures and stenting. During her hospital stay, she suffered multiple cardiac arrests due to respiratory failure and was not stabilized until airway tailored custom stent was inserted. She was later discharged with no significant long-term sequel. Conclusions It is important for the anesthesia staff, as part of the airway management plan team, to recognize the exact location and the anatomy of tracheomalacia. Certain anatomical deviations could affect our usual airway management pathways. Failure to do that can lead either to airway obstructions or detrimental hypoxia.

This case involved a 17-month-old toddler with Cohen's syndrome and cerebral palsy who had experienced multiple hospitalizations and operations since birth. During hospitalization, the patient suffered from tracheomalacia and poor swallowing and coughing abilities and had experienced two cardiopulmonary resuscitation events, after which a tracheotomy was suggested. Decision-making regarding the tracheotomy placed the mother under tremendous pressure, who was facing both the life-threatening disease of her son and her burden of care. During the nursing care period from 19th September to 6th December 2022, the author identified the main nursing problem as "decision-conflict: decision-making in relation to tracheotomy procedure" and distinguished the nursing process into (1) escape period, (2) conflict period, and (3) coping period after decision-making. Adopting the ten carative factors in Watson's Caring Theory as the core of her care approach, the author was able to establish a mutually trusting relationship with the mother and to understand her needs by means of listening, consistency, and empathy. This involved providing sufficient information and holding family meetings to facilitate communication in combination with palliative medical resources to provide follow-up care support as well as helping the family find meaning in their lives. [ABSTRACT FROM AUTHOR]

Objective: Posterior tracheomalacia (TM) is characterized by excessive intraluminal displacement of the tracheal membranous wall. Recently, novel surgical strategies for repair of posterior TM have been introduced. To our knowledge, these strategies have not been evaluated in a model of posterior TM. Thus, we sought to design an ex vivo mechanical model of posterior TM to evaluate potential repair interventions. Methods: A model for posterior TM was created with partial thickness longitudinal incisions to the posterior aspect of ex vivo porcine trachea. Three groups of tracheas were tested: (1) control (unmanipulated), (2) posterior TM (injury), and (3) intervention (repair). Interventions included external splinting with 0.3 and 0.5 mm bioresorbable plates, posterior tracheopexy, and injection tracheoplasty with calcium hydroxylapatite. An airtight tracheal system was created to measure tracheal wall collapse with changes in negative pressure. A bronchoscope and pressure transducer were connected to either end. Cross‐sectional area of the tracheal lumen was analyzed using ImageJ software (National Institutes of Health, Bethesda, MD). Results: Average percent reduction in cross‐sectional area of the tracheal lumen was compared using a two‐tailed paired t‐test. Significant differences were found between control and TM groups (p < 0.019). There was no significant difference between control and external splinting and posterior tracheopexy groups (p > 0.14). Conclusion: We describe an ex vivo model for posterior TM that replicates airway collapse. External splinting and tracheopexy interventions showed recovery of the injured tracheal segment. Injection tracheoplasty did not improve the TM. Level of Evidence: N/A Laryngoscope, 133:2000–2006, 2023 [ABSTRACT FROM AUTHOR]

Abstract Background Improved survival of neonates with esophageal atresia with/without tracheoesophageal fistula (EA/TEF) has resulted in increased prevalence of gastro-esophageal and respiratory-related morbidities. However, long-term outcome data on these patients remains limited, making it difficult to substantiate any guidelines on their chronic care. The purpose of our study is to report on their post-operative outcomes especially the long-term gastro-esophageal and respiratory morbidities. Methods This was a retrospective review of 65 patients (39 males, 26 females) who underwent EA/TEF repair from 1996 to 2019 at a single tertiary institution. Follow up data pertaining to clinical characteristics, operative management and long-term gastro-esophageal and respiratory morbidities was analyzed. Results Fifty-seven patients (87.7%) had Gross Type-C anatomy, followed by 5(7.7%) patients with Type-A, 1(1.5%) with Type-B and 1 with Type-D. One patient had a late diagnosis of H-type fistula (Type-E). Thirteen (20%) patients had long-gap EA. Median age at first surgery was day 1 (IQR 1–2) of life. All patients underwent bronchoscopy at their index surgery. All 52 non-long gap EA (LGEA) patients underwent primary anastomosis, while most (76.9%) LGEA patients underwent staged repair. Post-operatively, 4(6.2%) developed anastomotic leak which resolved with conservative management. Three (4.6%) had recurrent TEF, 2 underwent re-do ligation. Twenty (30.8%) patients developed anastomotic strictures, with 15 requiring serial dilatation. Long-term burden of gastro-esophageal and respiratory morbidity was high (63.1%; 64.6% respectively). The majority (n = 39,60%) of patients required active follow-up for a median duration of 5 years (IQR 1.5–10 years). Predominant conditions were gastroesophageal reflux disease (n = 28, 43.1%), dysphagia (n = 20, 30.8%), recurrent respiratory infections (n = 23, 35.3%), chronic cough (n = 19, 29.2%), and pneumonia (n = 19, 29.2%). Tracheomalacia was diagnosed in 22(33.8%), 2 of whom required tracheostomy for severe disease. Overall mortality rate was 10.8% (n = 7): 5 demised due to chronic respiratory failure, while 2 demised intra-operatively during the index surgery. Conclusion Despite successful surgical repair for EA/TEF, our data demonstrated significant morbidities among EA/TEF survivors, thus highlighting the importance of long-term multi-disciplinary care with collaboration between respiratory, gastroenterology, and otolaryngology specialists. Level of evidence Prognostic, Level IV.

Abstract Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. Respiratory morbidity related to external airways compression is a major concern in children affected by vascular rings. Clinical presentation depends on the severity of the tracheal lumen reduction and the presence of associated tracheomalacia. Recurrent respiratory infections, wheezing, atelectasis, and hyperinflation are mostly reported. As they are nonspecific and therefore difficult to recognize, attention should be given to all children with history of respiratory distress, extubation failure, noisy breathing, and recurrent respiratory infections. Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients.

Down Syndrome (DS) is the most common chromosomal abnormality compatible with life. The life of patients suffering from DS can be strongly impacted by Recurrent Respiratory tract Infections (RRIs), leading to an increased rate of hospitalisation, a higher need for intensive care and fatality. With a literature review, we summarise here the main etiological factors for RRI in this category of patients, particularly focusing on airway malformations such as tracheomalacia, tracheal bronchus and bronchomalacia, comorbidities associated with the syndrome, like congenital heart diseases, dysphagia, gastroesophageal reflux, musculoskeletal involvement and obesity, and immunologic impairments, involving both innate and adaptive immunity. For these patients, a multidisciplinary approach is imperative as well as some preventive strategies, in particular vaccinations in accordance with their national schedule for immunization.

This article discusses a case report of a collapse crisis of tracheomalacia caused by undiagnosed relapsing polychondritis (RP) during general anesthesia. RP is a rare multisystem disease characterized by recurrent inflammatory responses in cartilage tissue. The patient in this case was a 37-year-old male who experienced difficulty with tracheal intubation during surgery, leading to dyspnea and inspiratory wheezing. After further examination and consultation, the patient was diagnosed with RP and treated with oral glucocorticoids. The article emphasizes the importance of considering RP as a potential cause of subglottic stenosis and highlights the need for improved preoperative evaluation and management of difficult airway intubation in RP patients. [Extracted from the article]

Background: Tracheomalacia frequently develops in esophageal atresia patients (EA) especially in those with tracheoesophageal fistula (TEF). Conservative management has been the standard treatment; however, it was reported that delay in management could result in chronic lung conditions. Thus, early surgical interventions have been recently recommended. Patients and Methods: We enrolled patients presented with type-C esophageal atresia, with concomitant moderate to severe tracheomalacia, who had their thoracoscopic intervention done during the study period 2019–2022. Early and intermediate- term outcomes were studied and compared to another cohort with mild or no tracheomalacia. Results: During the allocated study period, 24 patients met the inclusion criteria for tracheopexy, but only 17 were followed up due to early demise of the other seven. During the follow-up period, 7/17 patients developed respiratory symptoms, which were attributed to esophageal stricture in 5 patients, recurrent TEF in 2 patients and one of them had residual tracheomalacia in addition to the developed recurrent TEF. The outcomes of the enrolled patients for tracheopexy were similar to those with no or mild tracheomalacia. Conclusions: Primary posterior tracheopexy during the primary repair might be considered a safe and feasible option not only to alleviate respiratory symptoms secondary to tracheomalacia in EA patients, but also to decline the need of further surgical interventions. However, more comprehensive studies with long-term follow-ups are mandatory.

Bronchopulmonary dysplasia (BPD), a disorder characterized by arrested lung development, is a frequent cause of morbidity and mortality in premature infants. Parenchymal lung changes in BPD are relatively well-characterized and highly studied; however, there has been less emphasis placed on the role that airways disease plays in the pathophysiology of BPD. In preterm infants born between 22 and 32 weeks gestation, the conducting airways are fully formed but still immature and therefore susceptible to injury and further disruption of development. The arrest of maturation results in more compliant airways that are more susceptible to deformation and damage. Consequently, neonates with BPD are prone to developing airway pathology, particularly for patients who require intubation and positive-pressure ventilation. Airway pathology, which can be divided into large and small airways disease, results in increased respiratory morbidity in neonates with chronic lung disease of prematurity. [ABSTRACT FROM AUTHOR]

Background: Members of anesthesia teams are involved in the management of COVID-19 infection cases at many stages particularly during acute airway interventions. Here, we describe a series of airway procedures to manage complex post-COVID-19 tracheomalacia in a high-risk patient. Case presentation: A 67-year-old female with a medical history of diabetes mellitus, hypertension, and morbid obesity and recent COVID-19 infection requiring prolonged intubation presented to our hospital in severe respiratory distress. She was found to have long-segment tracheomalacia requiring multiple airway surgical procedures and stenting. During her hospital stay, she suffered multiple cardiac arrests due to respiratory failure and was not stabilized until airway tailored custom stent was inserted. She was later discharged with no significant long-term sequel. Conclusions: It is important for the anesthesia staff, as part of the airway management plan team, to recognize the exact location and the anatomy of tracheomalacia. Certain anatomical deviations could affect our usual airway management pathways. Failure to do that can lead either to airway obstructions or detrimental hypoxia. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to describe the epidemiology of laryngotracheal stenosis within a resource-constrained setting, whilst exploring the outcome correlates unique to Montgomery Safe-T-Tube stented laryngotracheoplasty. Methods: A retrospective cross-sectional study of patients who underwent Montgomery Safe-T-Tube stented laryngotracheoplasty between January 2000 and December 2019 was performed. Results: Amongst 75 patients, most lesions were iatrogenic (78.7 per cent) and high-grade in severity (84 per cent). Following 101 laryngotracheoplasties, 57 patients (76 per cent) were successfully decannulated. Young age (84.6 per cent; p = 0.009), low-grade stenosis (100 per cent; p = 0.034) and airway-framework structural integrity (79.3 per cent; p = 0.004) were significant correlates of success. Restenosis (n = 43; 57.3 per cent), occurring at a median of 9.37 weeks following decannulation, was predominantly associated with antecedent dilatation (96.3 per cent; p < 0.001). Conclusion: Demographic and clinical profiles play a pivotal role in the outcomes and complications of Montgomery Safe-T-Tube stented laryngotracheoplasty. The success rate validates the procedure within a resource-limited setting. There exist critical periods following both surgery and decannulation when the occurrence of adverse events is most likely. [ABSTRACT FROM AUTHOR]

Introduction: There is currently no validated diagnostic modality to characterize the anatomy and predict outcomes of tracheal esophageal defects, such as esophageal atresia (EA) and tracheal esophageal fistulas (TEFs). We hypothesized that ultra-short echo-time MRI would provide enhanced anatomic information allowing for evaluation of specific EA/TEF anatomy and identification of risk factors that predict outcome in infants with EA/TEF. Methods: In this observational study, 11 infants had pre-repair ultra-short echo-time MRI of the chest completed. Esophageal size was measured at the widest point distal to the epiglottis and proximal to the carina. Angle of tracheal deviation was measured by identifying the initial point of deviation and the farthest lateral point proximal to the carina. Results: Infants without a proximal TEF had a larger proximal esophageal diameter (13.5 ± 5.1 mm vs. 6.8 ± 2.1 mm, p = 0.07) when compared to infants with a proximal TEF. The angle of tracheal deviation in infants without a proximal TEF was larger than infants with a proximal TEF (16.1 ± 6.1° vs. 8.2 ± 5.4°, p = 0.09) and controls (16.1 ± 6.1° vs. 8.0 ± 3.1°, p = 0.005). An increase in the angle of tracheal deviation was positively correlated with duration of post-operative mechanical ventilation (Pearson r = 0.83, p < 0.002) and total duration of post-operative respiratory support (Pearson r = 0.80, p = 0.004). Discussion: These results demonstrate that infants without a proximal TEF have a larger proximal esophagus and a greater angle of tracheal deviation which is directly correlated with the need for longer post-operative respiratory support. Additionally, these results demonstrate that MRI is a useful tool to assess the anatomy of EA/TEF. [ABSTRACT FROM AUTHOR]

Progressive corneal opacification can result from multiple etiologies, including corneal dystrophies or systemic and genetic diseases. We describe a novel syndrome featuring progressive epithelial and anterior stromal opacification in a brother and sister and their mildly affected father, with all three family members having sensorineural hearing loss and two also with tracheomalacia/laryngomalacia. All carried a 1.2 Mb deletion at chromosome 13q12.11, with no other noteworthy co-segregating variants identified on clinical exome or chromosomal microarray. RNAseq analysis from an affected corneal epithelial sample from the proband's brother revealed downregulation of XPO4, IFT88, ZDHHC20, LATS2, SAP18, and EEF1AKMT1 within the microdeletion interval, with no notable effect on the expression of nearby genes. Pathway analysis showed upregulation of collagen metabolism and extracellular matrix (ECM) formation/maintenance, with no significantly down-regulated pathways. Analysis of overlapping deletions/variants demonstrated that deleterious variants in XPO4 were found in patients with laryngomalacia and sensorineural hearing loss, with the latter phenotype also being a feature of variants in the partially overlapping DFNB1 locus, yet none of these had reported corneal phenotypes. Together, these data define a novel microdeletion-associated syndromic progressive corneal opacification and suggest that a combination of genes within the microdeletion may contribute to ECM dysregulation leading to pathogenesis. [ABSTRACT FROM AUTHOR]

Following surgical correction, many patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) present to the emergency department (ED) with acute airway complications. We sought to determine the incidence and risk factors for severe acute life-threatening events (ALTEs) in pediatric patients with repaired congenital EA/TEF and the outcomes of operative interventions. A retrospective cohort chart review was performed on patients with EA/TEF with surgical repair and follow-up at a single centre from 2000 to 2018. Primary outcomes included 5-year ED visits and/or hospitalizations for ALTEs. Demographic, operative, and outcome data were collected. Chi-square tests and univariate analyses were performed. In total, 266 EA/TEF patients met inclusion criteria. Of these, 59 (22.2%) had experienced ALTEs. Patients with low birth weight, low gestational age, documented tracheomalacia, and clinically significant esophageal strictures were more likely to experience ALTEs (p < 0.05). ALTEs occurred prior to 1 year of age in 76.3% (45/59) of patients with a median age at presentation of 8 months (range 0–51 months). Recurrence of ALTEs after esophageal dilatation was 45.5% (10/22), mostly due to stricture recurrence. Patients experiencing ALTEs received anti-reflux procedures (8/59, 13.6%), airway pexy procedures (7/59, 11.9%), or both (5/59, 8.5%) within a median age of 6 months of life. The resolution and recurrence of ALTEs after operative interventions are described. Significant respiratory morbidity is common among patients with EA/TEF. Understanding the multifactorial etiology and operative management of ALTEs have an important role in their resolution. Original Research, Clinical Research. Level III Retrospective Comparative Study. • The incidence of ALTEs in EA/TEF patients is 22.2% and they mostly occur before age 1-year. • ALTEs are associated with tracheomalacia, esophageal strictures, low birth weight, and low gestational age. • The long-term recurrence of ALTEs after esophageal dilatation, fundoplication, and airway pexy procedures are described. [ABSTRACT FROM AUTHOR]