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1.
Article in English | MEDLINE | ID: mdl-38960728

ABSTRACT

OBJECTIVES: The objective was to measure health-related quality of life (HRQoL) of children following treatment of all-cause tracheomalacia with aortopexy. METHODS: Children ≥5 years and parents of children <18 years who had undergone aortopexy completed the Paediatric Quality of Life Inventory (PedsQL4.0). Scores were compared to published norms. RESULTS: Completed questionnaires were received from 35 parents (65%) and 10 children (38%). Median age at aortopexy was 9.8 months (1 month-12.7 years) and median years of follow-up was 2.6 (4 months-6.9 years). Children who completed questionnaires had a median age of 8.4 (5.7-13.4) years. Parent and child-reported total PedsQL scores were 69.61 (SD : 19.74), and 63.15 (SD : 20.40) respectively. Half of parents and 80% of children reported scores suggesting poor HRQoL outcomes. Parent-reported total, physical and psycho-social scores were lower than those of healthy children and those with acute illness but comparable to children with chronic health conditions and cardiovascular disease. Similarly, children themselves reported comparable total scores to children with chronic illness but child-reported psycho-social scores were lower in the aortopexy group than any other group. There was no association between PedsQL scores and cause of malacia, age or time since aortopexy. The presence of complex congenital comorbidities had a significant (p < 0.05) impact on HRQoL scores. CONCLUSIONS: Following aortopexy children remain at risk of poor HRQoL, especially those with complex comorbidities. HRQoL reported by both parent and child provides important insight into the lives of children following this procedure. Further longitudinal and qualitative study are required to better understand this complex group.

2.
J. pediatr. (Rio J.) ; 100(3): 250-255, May-June 2024. tab
Article in English | LILACS-Express | LILACS | ID: biblio-1558320

ABSTRACT

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.

3.
Respirol Case Rep ; 12(5): e01348, 2024 May.
Article in English | MEDLINE | ID: mdl-38690063

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.

4.
Rev Mal Respir ; 41(6): 421-438, 2024 Jun.
Article in French | MEDLINE | ID: mdl-38762394

ABSTRACT

Relapsing polychondritis is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the cartilages involved. In addition to characteristic damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are particularly severe, and can seriously alter the prognosis. Tracheobronchial lesions are assessed by means of a multimodal approach, including dynamic thoracic imaging, measurement of pulmonary function (with recent emphasis on pulse oscillometry), and mapping of tracheal lesions through flexible bronchoscopy. Diagnosis can be difficult in the absence of specific diagnostic tools, especially as there may exist a large number of differential diagnoses, particularly as regards inflammatory diseases. The prognosis has improved, due largely to upgraded interventional bronchoscopy techniques and the development of immunosuppressant drugs and targeted therapies, offering patients a number of treatment options.


Subject(s)
Bronchial Diseases , Polychondritis, Relapsing , Polychondritis, Relapsing/diagnosis , Polychondritis, Relapsing/complications , Humans , Diagnosis, Differential , Bronchial Diseases/diagnosis , Bronchial Diseases/pathology , Bronchial Diseases/etiology , Tracheal Diseases/diagnosis , Tracheal Diseases/pathology , Bronchoscopy/methods , Trachea/pathology , Bronchi/pathology
5.
Pediatr Pulmonol ; 2024 May 09.
Article in English | MEDLINE | ID: mdl-38721899

ABSTRACT

INTRODUCTION: The aims of this study were to update our experience with biodegradable polydioxanone (PDO) airway stents in children, focusing on effectiveness and safety, and to analyze the factors involved in the different outcomes observed. MATERIALS AND METHODS: Retrospective study of patients managed with PDO stents from 2012 to 2023. Variables collected: demographics, comorbidities, indication, clinical baseline, stent size, location, complications, clinical outcome, and time of follow-up. Statistical analyses were performed to detect the eventual contribution of variables in the different outcomes observed. RESULTS: Fifty-four PDO stents were placed in 26 patients (median age, 4 months). All showed severe symptoms of central airway obstruction due to tracheomalacia in nine patients, bronchomalacia five, tracheobronchomalacia 10, and tracheal stenosis two. Stent placement was uneventful in every case: 29 stents in the trachea and 25 in the main bronchi. 53.8% of patients needed successive stenting, and all exhibited comorbidities. Complete clinical resolution was observed in eight cases (30.7%), partial improvement in 13 (50%), unchanged in 4 (15.3%), and worsened in one. Age had a significant positive impact on outcome (6 vs. 3 months; p = 0.024). Additionally, smaller stents were associated with a better outcome (20 vs. 26 mm; p = 0.044). Granulation tissue was the most frequent complication (34.6%). Five patients (19.2%) died due to severe comorbidities, follow-up was complete in survivors (median, 58 months). CONCLUSIONS: PDO stents are safe and effective when dealing with severe tracheobronchial obstruction. Stent-related granulation tissue continues to be a relevant matter of concern. This issue, together with increased degradation times, deserves further research.

6.
Cureus ; 16(3): e56875, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38659555

ABSTRACT

Endotracheal cuff-pressure monitoring is a critical component of patient care in the intensive care unit, ensuring the safety and efficacy of mechanical ventilation. Despite its importance, there remains a lack of standardized protocols regarding optimal pressure targets and documentation practices. This editorial examines the significance of endotracheal intracuff-pressure monitoring in enhancing patient outcomes, highlighting the challenges and potential solutions in clinical practice.

7.
Pediatr Pulmonol ; 2024 Apr 17.
Article in English | MEDLINE | ID: mdl-38629381

ABSTRACT

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.

8.
HNO ; 2024 Apr 03.
Article in German | MEDLINE | ID: mdl-38568240

ABSTRACT

BACKGROUND: Suprastomal collapse (SSC) is considered a major late complication of paediatric tracheostomy and can be responsible for decannulation failure in up to 20% of tracheostomised children. Depending on the severity of SSC, surgery may be required. Various strategies and techniques are available, of which the treating with airway team should be aware. OBJECTIVE: This article intends to summarise the aetiology of SSC, its classification, clinical presentation, and the gold standard diagnostic and therapeutic algorithms according to the current literature. MATERIALS AND METHODS: A panel of experts reviewed the available literature on SSC. Published evidence on the different surgical techniques and their advantages and disadvantages was reviewed in detail, and a treatment algorithm created. RESULTS: The gold standard diagnostic procedure for SSC is flexible transnasal laryngotracheoscopy in spontaneous breathing followed by microlaryngoscopy (MLS) under general anaesthesia. Two main types of SSC can be differentiated, which differ in terms of surgical treatment. Purely anterior SSC is usually treated by tracheoplasty using an anterior costal cartilage graft (ACCG). Simple closure of the tracheostomy or excision of SSC with a potassium-titanyl-phosphate (KTP) laser are also described as less invasive approaches. For anterolateral SSC, segmental tracheal resection with end-to-end anastomosis or tracheoplasty with ACCG represent promising treatment options. Tracheal reinforcement with absorbable microplates is also discussed in the literature. With both types of SSC and depending on severity and the age of the child, a watch-and-wait strategy should always be considered. CONCLUSION: Dynamic airway endoscopy in spontaneous breathing followed by MLS in general anaesthesia should always be performed before decannulation. It is particularly important to visualise all segments of the airway during spontaneous breathing. The decision regarding the best surgical option for each child is based on the type and localisation of SSC, as well as on the patient's medical and surgical history and age.

9.
J Pediatr Surg ; 59(6): 1066-1071, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38429129

ABSTRACT

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Subject(s)
Esophageal Atresia , Tracheoesophageal Fistula , Humans , Tracheoesophageal Fistula/surgery , Tracheoesophageal Fistula/complications , Esophageal Atresia/surgery , Esophageal Atresia/complications , Retrospective Studies , Infant, Newborn , Male , Female , Follow-Up Studies , Infant , Treatment Outcome , Child, Preschool , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Laryngostenosis/surgery , Recurrence , Larynx/abnormalities , Larynx/surgery , Congenital Abnormalities
10.
Children (Basel) ; 11(2)2024 Feb 15.
Article in English | MEDLINE | ID: mdl-38397357

ABSTRACT

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.

11.
Int J Surg Case Rep ; 116: 109211, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38310789

ABSTRACT

INTRODUCTION: Nontoxic nodular goiter is one of the most prevalent thyroid conditions worldwide. Thyroidectomy for large goiters has a relatively high risk of postoperative airway obstruction, with tracheomalacia being one of the potential complications. CASE REPORT: A 61-year-old woman complained of a lump in her neck for 45 years. The node is progressively enlarged, but she did not experience any breathing difficulty, hoarseness, or pain while swallowing. A total thyroidectomy was then performed. The histopathologic examination revealed colloid goiter. During the procedure, evaluation of the trachea revealed a tracheomalacia, so a tracheotomy was then performed on the patient. After a follow-up period of three months, the patient was no longer experiencing tracheomalacia, and the tracheostomy was successfully closed. DISCUSSION: Surgery has been considered an acceptable approach for managing non-toxic goiter. The most common indications are compressive symptoms, substernal extension, inability to control hyperthyroidism through medication, and a suspicion of malignancy. However, thyroidectomy for large goiter carries a relatively high risk of postoperative respiratory obstruction. Diagnosing tracheomalacia can be challenging and often relies on bronchoscopy to assess the airway and observe the collapse of cartilage and membranes. Acquired tracheomalacia can be managed through internal or external stenting or tracheostomy. CONCLUSION: Total thyroidectomy has been recommended as a suitable procedure for non-toxic and toxic multinodular goiter. Tracheomalacia may occur following thyroidectomy in patients with thyroid enlargement. Tracheostomy effectively manages tracheomalacia by creating a channel across the malacia's focal segment, restoring the airway's patency.

12.
Pediatr Pulmonol ; 59(4): 899-906, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38197524

ABSTRACT

INTRODUCTION: Tracheomalacia (TM) is an important cause of respiratory morbidity. Dynamic flexible bronchoscopy is considered the gold standard for diagnosis. Dynamic airway computed tomography (DACT) is a low radiation, noninvasive diagnostic tool utilizing images obtained continuously over several respiratory cycles. We aimed to assess the accuracy of DACT in TM diagnosis. METHODS: Retrospective analysis of all patients who underwent both DACT and flexible bronchoscopy within 6 months. Airway anterior-posterior (AP) diameter was measured on multiplanar reconstructions CT in both the inspiratory and expiratory phases. Using still images from the bronchoscopy videos, the AP diameter of the trachea was measured at points of maximal and minimal diameter during tidal breathing. Degree of TM on both DACT and flexible bronchoscopy were graded using a scaling system of 50%-74%, 75%-89%, and 90%-100% as described by the European Respiratory Society. RESULTS: Twenty-four patients met inclusion criteria with an average time of 19.5 days between CT and bronchoscopy. The specificity and sensitivity of DACT for the overall diagnosis of TM was 100% and 68%, respectively, with a positive predictive value of 100% and a negative predictive value of 62%. There was a strong positive correlation between DACT and flexible bronchoscopy in the measurement of tracheal AP diameter changes (ρ = 0.773, R2 0.597, p = 0.00001). Mean effective radiation dose for DACT was 0.1 mSv. CONCLUSION: Ultralow dose DACT has excellent specificity and positive predictive value for both detection of TM and categorizing severity of tracheal collapse but is not sufficiently sensitive to rule it out.


Subject(s)
Tracheomalacia , Child , Humans , Tracheomalacia/diagnostic imaging , Bronchoscopy/methods , Retrospective Studies , Trachea/diagnostic imaging , Tomography, X-Ray Computed/methods
13.
J Pediatr (Rio J) ; 100(3): 250-255, 2024.
Article in English | MEDLINE | ID: mdl-38278512

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.


Subject(s)
Tracheobronchomalacia , Humans , Tracheobronchomalacia/surgery , Tracheobronchomalacia/complications , Retrospective Studies , Female , Male , Infant , Treatment Outcome , Infant, Newborn , Child, Preschool , Bronchoscopy , Severity of Illness Index , Child , Follow-Up Studies
14.
J Pediatr Surg ; 59(6): 1222-1227, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38184432

ABSTRACT

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.


Subject(s)
Esophageal Atresia , Esophageal Atresia/surgery , Esophageal Atresia/history , Humans , History, 20th Century , Esophagus/surgery , Infant, Newborn , History, 21st Century , Esophagoplasty/methods , Esophagoplasty/history
15.
Surg Today ; 54(3): 211-219, 2024 Mar.
Article in English | MEDLINE | ID: mdl-36729255

ABSTRACT

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.


Subject(s)
Esophageal Atresia , Esophagoplasty , Tracheomalacia , Humans , Infant , Tracheomalacia/surgery , Tracheomalacia/complications , Esophageal Atresia/surgery , Trachea/surgery , Treatment Outcome
16.
Pediatr Pulmonol ; 59(2): 342-347, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37937891

ABSTRACT

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.


Subject(s)
Bronchomalacia , Tracheobronchomalacia , Humans , Child , Infant , Retrospective Studies , Trisomy 13 Syndrome , Trisomy 18 Syndrome , Bronchomalacia/diagnosis , Bronchomalacia/epidemiology , Bronchoscopy
17.
J Pediatr Surg ; 59(2): 197-201, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37949688

ABSTRACT

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.


Subject(s)
Tracheobronchomalacia , Tracheomalacia , Humans , Child , Infant , Retrospective Studies , Aorta/surgery , Tracheobronchomalacia/surgery , Tracheomalacia/surgery , Sternotomy/adverse effects , Sternotomy/methods
18.
J Pediatr Surg ; 59(1): 10-17, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37903674

ABSTRACT

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.


Subject(s)
Esophageal Atresia , Tracheobronchomalacia , Tracheoesophageal Fistula , Infant , Infant, Newborn , Humans , Esophageal Atresia/surgery , Esophageal Atresia/complications , Treatment Outcome , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Postoperative Complications/prevention & control , Tracheoesophageal Fistula/surgery , Tracheoesophageal Fistula/complications , Tracheobronchomalacia/complications , Morbidity , Retrospective Studies
19.
J Aerosol Med Pulm Drug Deliv ; 37(1): 19-29, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38064481

ABSTRACT

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.


Subject(s)
Lung Diseases , Tracheomalacia , Infant, Newborn , Infant , Humans , Administration, Inhalation , Lung , Trachea , Particle Size , Respiratory Aerosols and Droplets
20.
Paediatr Anaesth ; 34(4): 289-292, 2024 04.
Article in English | MEDLINE | ID: mdl-38130114

ABSTRACT

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.


Subject(s)
Anesthetics , Tracheobronchomalacia , Humans , Child , Suction , Tracheobronchomalacia/surgery , Respiration , Airway Extubation
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