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.

[Pediatric tracheostomy: Ten year experience in an Intensive Care Unit]. / Traqueostomía en niños: Experiencia de 10 años en una Unidad de Cuidados Intensivos Pediátricos.

INTRODUCTION: Pediatric tracheostomy indications have changed over the last 30 years, from acute and transient pro cedures secondary to airway obstruction to programmed tracheostomies indicated due to the need for chronic use of mechanical ventilation (MV). OBJECTIVE: To describe indications and morbidity associated with pediatric tracheostomies during a ten-year period. PATIENTS AND METHODS: Descrip tive study. Clinical records review of discharged patients (< 15 years old) tracheostomized during their hospital stay between 2005 and 2015. Demographic and clinical variables were evaluated before and after tracheostomy, stay in intensive care unit, age at the time of the tracheostomy, indication of tracheostomy, early complications (< 7 days), late complications (> 7 days), and mortality. RESULTS: 59 children with tracheostomy were analyzed, 36 (59%) tracheostomies were performed in children under 6 months, and 39 (60%) in males. 23 (39%) had a confirmed or under study genopathy and 25 (42%) had congenital heart disease. The main indications for tracheostomy were 58% secondary to airway disease and 42% due to chronic use of MV. Within the airway disease group, subglottic steno sis, vocal cord paralysis, and tracheobronchomalacia were the principal reasons for indication, and in the group of chronic use of MV, the main causes were bronchopulmonary dysplasia and chronic lung disease. We did not find tracheostomy-related mortality. 89% of the patients were discharged with tracheostomy and 59% with chronic use of MV. The probability of being discharged with a tracheos tomy was higher in younger patients while the chronic use of MV at discharge was higher in patients with a greater number of extubation failures before tracheostomy. CONCLUSION: Tracheostomy is a safe procedure in children, where the predominant causes of indication are airway disease and the need for chronic use of MV. Most children with tracheostomies are discharged with tracheostomy and chronic use of MV. Younger children, those with difficult weaning, confirmed or suspected ge nopathy, or special health needs are at greater risk of needing tracheostomy and chronic use of MV.

Clinical survey and predictors for the development of tracheobronchomalacia in preterm infants.

BACKGROUND: Tracheobronchomalacia (TBM) contributes to the increased morbidity and mortality observed in preterm infants. Effective strategies for the prevention of TBM are necessary to achieve better outcomes. We sought to identify risk factors associated with the development of TBM in preterm infants. Optimal cut-off values for each risk factor were also determined. METHODS: A total of 80 infants who were born at 36 week's gestation or earlier and underwent flexible bronchoscopy were included in our study sample. A comparison of demographic and clinical risk factors between those with TBM (n = 35, 44%) and those without TBM (n = 45, 56%) was conducted using multivariate logistic regression analysis. Receiver operating characteristic curve analysis was performed to determine the appropriate cut-off values for predicting the development of TBM. RESULTS: In the multivariate analysis, only peak inspiratory pressure (PIP) and the number of intubation days remained significantly different between infants with and without TBM. Preterm infants with TBM received higher PIP (odds ratio: [OR], 1.067; 95% confidence interval [CI], 1.010-1.128; p = .020) and were intubated for longer (odds ratio [OR], 1.019; 95% CI, 1.003-1.035; p = .016) than those without TBM. Infants who received PIP > 19.5 cmH2 O or were intubated for >79.5 days were associated with a significantly higher risk of presence of TBM. CONCLUSION: High PIP and prolonged intubation were major risk factors for the development of TBM in premature infants. Those who require PIP > 19.5 cmH2 O or intubation >79.5 days warrant bronchoscopy examination for early diagnosis and management of TBM.

Great vessel anomalies and their impact on the surgical treatment of tracheobronchomalacia.

BACKGROUND: Tracheobronchial compression (TBC) from great vessel anomalies (GVA) can contribute to tracheobronchomalacia (TBM) symptoms. The frequency, impact on symptoms and optimal management of GVA in these patients, with or without a history of esophageal atresia (EA), are still unclear. STUDY DESIGN: Patients who underwent surgery for TBM/ TBC between 2001 and 2017 were reviewed. Demographics, type of GVA, and operative interventions were collected. The frequency and treatment modalities of GVA between EA and non-EA patients were compared. RESULTS: Overall, 209 patients met criteria; 120 (57.4%) patients had at least one GVA, including double aortic arches (n = 4, 1.9%), right aortic arches (n = 14, 6.7%), aberrant right subclavian arteries (n = 15, 7.2%), and innominate artery compression (n = 71, 34.0%). Non-EA patients were more likely to have surgery later in life (29.5 months versus 16 months, p = 0.0002), double aortic arch (p = 0.0174), right aortic arch (p < 0.0001), and undergo vascular reconstruction concurrently with their airway procedure (25% vs 8.4%, p = 0.002). Vessel reconstruction was performed in 25 patients; six required cardiac bypass. CONCLUSION: The frequency of GVA in patients with symptomatic airway collapse is substantial. Multidisciplinary evaluation is imperative for operative planning as many require complex reconstruction and collaboration with cardiac surgery, particularly patients without a history of EA. LEVEL OF EVIDENCE: Level III.

Excessive Dynamic Airway Collapse or Tracheobronchomalacia: Does It Matter? / Colapso dinámico excesivo de la vía aérea o traquebroncomalacia: ¿es relevante?

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The Prevalence of Gastroesophageal Reflux in Patients With Excessive Central Airway Collapse.

BACKGROUND: Gastroesophageal reflux (GER) is increasingly recognized as an exacerbating or causal factor in several respiratory diseases. There is a high prevalence of GER in infants with airway malacia. However, such data are lacking in adults. METHODS: This retrospective study was conducted to determine the relationship between GER and excessive central airway collapse (ECAC). The study included consecutive patients with ECAC referred to the Complex Airway Center at Beth Israel Deaconess Medical Center who underwent esophageal pH testing for GER between July 2014 and June 2018. RESULTS: Sixty-three of 139 patients with ECAC (45.3%) had documented GER as shown by an abnormal esophageal pH test result. The mean DeMeester score was 32.2, with a symptom association probability of 39.7% of GER-positive patients. Twenty-nine of 63 patients (46%) with GER reported improvement in respiratory symptoms following maximal medical therapy or antireflux surgery without requiring further treatment for ECAC. CONCLUSIONS: GER is prevalent among patients with ECAC, and aggressive reflux treatment should be considered in these patients prior to considering invasive airway procedures or surgery.

Aortopexy for the treatment of tracheobronchomalacia in 100 children: a 10-year single-centre experience.

OBJECTIVES: Our study describes and analyses the results from aortopexy for the treatment of airway malacia in children. METHODS: Demographic data, characteristics and preoperative, operative and outcome details, including the need for reintervention, were collected for children undergoing aortopexy between 2006 and 2016. RESULTS: One hundred patients [median age 8.2 months, interquartile range (IQR) 3.3-26.0 months] underwent aortopexy. Sixty-four (64%) patients had tracheomalacia (TM) only, 24 (24%) patients had TM extending into their bronchus (tracheobronchomalacia) and 11 (11%) patients had bronchomalacia. Forty-one (41%) children had gastro-oesophageal reflux disease, of which 17 (41%) children underwent a Nissen fundoplication. Twenty-eight (28%) children underwent a tracheo-oesophageal fistula repair prior to aortopexy (median 5.7 months, IQR 2.9-17.6 months). The median duration of follow-up was 5.3 years (IQR 2.9-7.5 years). Thirty-five (35%) patients were on mechanical ventilatory support before aortopexy. Twenty-seven (77%) patients could be safely weaned from ventilator support during the same admission after aortopexy (median 2 days, IQR 0-3 days). Fourteen patients required reintervention. Overall mortality was 16%. Multivariable analysis revealed preoperative ventilation (P = 0.004) and bronchial involvement (P = 0.004) to be adverse predictors of survival. Only bronchial involvement was a predictor for reintervention (P = 0.012). CONCLUSIONS: Aortopexy appears to be an effective procedure in the treatment of children with severe airway malacia. Bronchial involvement is associated with adverse outcome, and other procedures could be more suitable. For the treatment of severe airway malacia with isolated airway compression, we currently recommend aortopexy to be considered.

Trans-nasal flexible bronchoscopy in wheezing children: Diagnostic yield, impact on therapy, and prevalence of laryngeal cleft.

AIM: Persistent or recurrent wheezing is a common indication for flexible bronchoscopy, as anatomic and infectious or inflammatory changes are highly prevalent. We sought to evaluate the prevalence of anatomic, infectious, and inflammatory disease in a cohort of children undergoing flexible bronchoscopy for wheezing or poorly controlled asthma. METHODS: We retrospectively reviewed all children <18 years old who underwent flexible bronchoscopy at our center from October 29, 2012-December 31, 2016 for the primary or secondary indication of wheezing (persistent, frequently recurring, or atypical) or poorly controlled asthma. RESULTS: A total of 101 procedures were identified in 94 patients, aged 3 months to 18 years. Potential anatomic causes for wheezing identified in 45.7% of patients and inflammatory changes in 49.5% of procedures. This included the identification of a laryngeal cleft in 17% for which half required medical or surgical management. Tracheobronchomalacia was the most commonly identified anatomic lesion. Thirty children from this cohort had poorly controlled asthma. Among this subgroup, 54% had increased neutrophils on BAL and 30% had an anatomic contributor to wheezing, including one with a laryngeal cleft. Based on findings from flexible bronchoscopy, management changes made in 63.8% of patients. This included medication changes in 54 and surgical intervention in 9. DISCUSSION: We conclude that transnasal flexible bronchoscopy has high yield in children with recurrent, persistent, or atypical wheezing and those with poorly controlled asthma. Laryngeal cleft has a reasonably high prevalence that warrants specific evaluation in this population.

Tracheobronchomalacia diagnosed by tracheobronchography in ventilator-dependent infants.

BACKGROUND: Tracheobronchomalacia prevalence in premature infants on prolonged mechanical ventilation is high. OBJECTIVE: To examine the prevalence of tracheobronchomalacia diagnosed by tracheobronchography in ventilator-dependent infants, and describe the demographic, clinical and dynamic airway characteristics of those infants with tracheobronchomalacia. MATERIALS AND METHODS: This retrospective review studies 198 tracheobronchograms performed from 1998 to 2011 in a cohort of 158 ventilator-dependent infants <2 years of age. Dynamic airway assessment during tracheobronchography determined the optimal positive end-expiratory pressure to maintain airway patency at expiration in those infants with tracheobronchomalacia. RESULTS: Tracheobronchograms were performed at a median age of 52 weeks post menstrual age. The primary diagnoses in these infants were bronchopulmonary dysplasia (53%), other causes of chronic lung disease of infancy (28%) and upper airway anomaly (13%). Of those with bronchopulmonary dysplasia, 48% had tracheobronchomalacia. Prematurity (P=0.01) and higher baseline - pre-tracheobronchogram positive end-expiratory pressure (P=0.04) were significantly associated with tracheobronchomalacia. Dynamic airway collapse during tracheobronchography showed statistically significant airway opening at optimal positive end-expiratory pressure (P < 0.001). There were no significant complications noted during and immediately following tracheobronchography. CONCLUSION: The overall prevalence of tracheobronchomalacia in this cohort of ventilator-dependent infants is 40% and in those with bronchopulmonary dysplasia is 48%. Infants born prematurely and requiring high pre-tracheobronchogram positive end-expiratory pressure were likely to have tracheobronchomalacia. Tracheobronchography can be used to safely assess the dynamic function of the airway and can provide the clinician the optimal positive end-expiratory pressure to maintain airway patency.

Tracheobronchomalacia in pediatric patients with esophageal atresia: comparison of diagnostic laryngoscopy/bronchoscopy and dynamic airway multidetector computed tomography.

PURPOSE: Diagnostic laryngoscopy and bronchoscopy (DLB) has been the traditional preoperative diagnostic modality for evaluating presence and severity of tracheobronchomalacia (TBM), and requires anesthesia. Alternatively, multidetector computed tomography (MDCT) is potentially a noninvasive modality that provides high-resolution, 3-dimensional (3D) imaging of the thorax providing preoperative guidance for pediatric surgeons. This study compares MDCT with intraoperative DLB in the assessment of TBM in symptomatic pediatric patients with esophageal atresia (EA). METHODS: Following IRB approval all pediatric patients (≤18 years) who had EA and who underwent an MDCT study as a preoperative evaluation of TBM prior to aortopexy were retrospectively reviewed. Patients with incomplete reports on intraoperative DLB or MDCT studies were excluded. Two pediatric radiologists independently evaluated all MDCT studies in a blinded fashion. On both DLB and MDCT studies, TBM was scored as present or absent in five anatomic segments: upper, middle, and lower trachea, as well as right and left main stem bronchi. Operative reports including DLB findings were reviewed and compared to findings from MDCT study using the chance corrected kappa (κ) coefficient. Diagnostic accuracy of dynamic MDCT for detecting TBM was determined by sensitivity and specificity, and interobserver agreement between two radiology reviewers was measured by the kappa statistic. RESULTS: The final study population included 18 patients (8 males and 10 females) with ages ranging from 1month to 11years (median: 7 months). Their presenting clinical symptoms included apneic spells (n=15, 83%) and failure to extubate (n=3, 17%). The overall diagnostic accuracy of dynamic airway MDCT compared to DLB was 91% (82/90 possible segments for TBM) with excellent overall agreement across all 5 anatomic segments (κ=0.82, p<0.001). The agreements for upper, mid, lower trachea, and right and left trachea were 89% (κ=0.73, p<0.001), 94% (κ=0.85, p<0.001), 89% (κ=0.76, p<0.001), 94% (κ=0.82, p<0.001), and 89% (κ=0.61, p=0.005); respectively. Interobserver agreement between two radiologists was excellent (κ=0.98, 95% confidence interval: 0.94-1.00, p<0.001) with only 1 disagreement between two radiologists that was found for the left main bronchus. Fifteen (83.3%) of the patients clinically improved after the aortopexy. CONCLUSION: MDCT with 3D imaging is a highly accurate and reliable preoperative noninvasive imaging modality for evaluating TBM in pediatric patients with EA providing anatomic information consistent with and complimentary to bronchoscopy.

Tracheomalacia/Tracheobronchomalacia and hyperdynamic airway collapse.

Tracheobronchomalacia (TBM) and hyperdynamic airway collapse (HDAC) can be debilitating diseases associated with decreased functional capacity and poor quality of life, although there is no standard definition of this complex condition, and there are numerous terms used to describe it. The diverse etiology associated with TBM and HDAC can obscure and delay an accurate diagnosis for years. A thorough medical history is important in understanding possible causes and in guiding diagnostic testing. Medical history may also suggest what treatments may be most beneficial.

Traqueobroncomalacia en niños / Tracheobronchomalacia in children

Tracheobronchomalacia refers to the presence of trachea and bronchi with soft, collapsible walls. Its incidence has been reported from 1:1500 to 1:2500. It can be congenital or acquired. This disease ranges from mild to life threatening. A high clinical suspicion is required for diagnosis, which needs to be confirmed endoscopically. Depending on severity, treatment can include from physical therapy and antibiotics to mechanical ventilation, surgery and tracheostomy, and is based on expert opinion and case series. Prognosis is usually good, with tendency to spontaneous resolution near the 2d year of life.

Traquebroncomalacia se refiere a la presencia de tráquea y/o bronquios cuya pared es blanda y tiene tendencia al colapso. Se ha reportado una incidencia entre 1:1.500 a 1:2.500. Puede ser congénita o adquirida. La severidad de los síntomas varía desde leve a incluso riesgo vital y muerte. El diagnóstico requiere una alta sospecha clínica y confirmación endoscópica. El tratamiento está basado en opiniones de expertos y series clínicas y varía según la severidad de los síntomas desde kinesioterapia y antibióticos en los casos más leves hasta ventilación mecánica, traqueostomía y cirugía en los más severos. El pronóstico en general es bueno con tendencia a la resolución espontánea aproximadamente a los 2 años de vida.

Tracheobronchomalacia in adults.

Severe, diffuse tracheobronchomalacia (TBM) is an underrecognized cause of dyspnea, recurrent respiratory infections, cough, secretion retention, and even respiratory insufficiency. Patients often have comorbidities, such as asthma or chronic obstructive pulmonary disease, and inappropriate treatment for these conditions may precede eventual recognition of TBM by months or years. Most of these patients have an acquired form of TBM in which the etiology in unknown. Diagnosis of TBM is made by airway computed tomography scan and flexible bronchoscopy with forced expiration. The prevailing definition of TBM as a 50% reduction in cross-sectional area is nonspecific, with a high proportion of healthy volunteers meeting this threshold. The clinically significant threshold is complete or near-complete collapse of the airway. Airway stenting may treat TBM, although complications resulting from indwelling prostheses often limit the durability of stents. Surgical stabilization of the airway by posterior splinting (tracheobronchoplasty) effectively and permanently corrects malacic airways. Proper surgical selection is facilitated by a short-term stent trial.

Congenital cystic adenomatoid malformation of lung type 1.

Congenital cystic adenomatoid malformation, a rare developmental, hamartomatous abnormality of the lung, usually is unilateral, is localized, and presents in early infancy. Delayed occurrence in older children and multilobar involvement are rare. We describe a case of congenital cystic adenomatoid malformation type 1 with multilobar involvement, associated emphysema, and coexistent tracheobronchopathia osteochondroplastica in an adolescent girl for whom the correct diagnosis was achieved only on histologic examination. The importance of an accurate diagnosis of this entity enables proper subtyping, management to minimize the risk of infections and malignancy, and exclusion of associated malformations.

Incidence of tracheobronchomalacia associated with pulmonary emphysema: detection with paired inspiratory-expiratory multidetector computed tomography using a low-dose technique.

PURPOSE: The purpose of this study was to evaluate the frequency of tracheobronchomalacia (TBM) associated with pulmonary emphysema with paired inspiratory-expiratory multidetector computed tomography (MDCT) using a low-dose technique. MATERIALS AND METHODS: This study included 56 consecutive patients (55 men, 1 woman; mean age 68.9 years) with pulmonary emphysema who had undergone paired inspiratory-expiratory CT scanning with a low-dose technique (40 mA). All images were retrospectively examined by two thoracic radiologists in a blinded fashion. The diagnosis of TBM was based on the standard criterion of >50% reduction in the cross-sectional area of the tracheobronchial lumen at the end-expiratory phase. A mild TBM criterion of >30% reduction was also reviewed. All patients underwent pulmonary function tests. The relation between the forced expiratory volume in 1 s (FEV(1.0%)) and TBM was statistically analyzed. RESULTS: Four (7.1%) and eight (14.3%) patients were diagnosed as TBM based on the standard and mild criteria, respectively. In four patients, the percentages of luminal narrowing were 63.4% and 51.2%, respectively for tracheomalacia and 59.2% and 62.0%, respectively, for bronchomalacia. The FEV(1.0%) values between patients with and without TBM showed no statistical difference. CONCLUSION: The incidence of TBM associated with pulmonary emphysema was 7.1% with the standard criterion. It is possible that TBM has been underdiagnosed in a number of patients with pulmonary emphysema.

Synchronous airway lesions and outcomes in infants with severe laryngomalacia requiring supraglottoplasty.

OBJECTIVE: To quantify the prevalence and the impact of synchronous airway lesions identified by endoscopy in infants undergoing supraglottoplasty for severe laryngomalacia (LM). DESIGN: Retrospective study. SETTING: Tertiary care pediatric hospital. PATIENTS: Sixty patients who underwent supraglottoplasty for severe LM from 2002 to 2006. Patients who underwent preoperative tracheotomy, had previous airway surgery, or did not have 6 months of follow-up were excluded. Fifty-two patients met inclusion criteria. INTERVENTION: Supraglottoplasty (with carbon dioxide laser). MAIN OUTCOME MEASURES: Presence of synchronous airway lesions and their contribution to upper airway obstruction (UAO) and their effect on the postoperative course after supraglottoplasty. RESULTS: Fifty-eight percent of patients had synchronous airway lesions (SALs), of whom 77% had subglottic stenosis (SGS) and 47% had tracheomalacia, bronchomalacia, or both. Sixty-three percent of all patients required postoperative nonsurgical airway support. Eight patients had residual UAO requiring additional surgical intervention, with 3 revision supraglottoplasties and 7 tracheotomies performed. Infants with neurological conditions had a high rate of surgical intervention (55%; P = .001). Patients with SGS exceeding 35% but without any neurological condition had a prolonged hospital stay (>3.6 days; P = .02) and an 83% incidence (P = .04) of postoperative UAO requiring intubation. Infants with LM with laryngeal edema (LE) alone had increased frequency of postoperative nonsurgical airway support (P = .02) and a prolonged hospital stay of 1 day (P = .01) compared with infants without edema. CONCLUSIONS: There is a high incidence of SALs in patients undergoing supraglottoplasty. Neurological conditions, hypoplastic mandible, SGS greater than 35%, and preexisting LE independently adversely affected the postoperative course.