Prevalence and Clinical Characteristics of Pediatric Lower Airway Malacia: Case Series from a Tertiary Center in Turkey.

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.

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

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

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

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

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

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

Clinicopathologic features, comorbid diseases, and prevalence of pulmonary hypertension in dogs with bronchomalacia.

BACKGROUND: Reports of clinicopathologic features of bronchomalacia (BM) differ because of inconsistent definitions and frequent prevalence of comorbid cardiopulmonary disease. Pulmonary hypertension (PH) secondary to BM is poorly described. OBJECTIVES: Dogs with BM will be older but of any somatotype, and increased expiratory effort, ≥1 comorbid disease, and PH will be more common than in dogs without BM. ANIMALS: Client-owned dogs (n = 210) evaluated for respiratory signs. METHODS: Medical records of dogs with paired inspiratory: expiratory-breath-hold computed tomography, tracheobronchoscopy, or both between January 2016 and December 2019 were retrospectively reviewed. Comparisons between dogs with and without BM using Mann-Whitney rank sum or χ2 tests (P < .05 significant were made). Because of high numbers of variables, criteria with high prevalence (>25%) were identified (n = 10) for univariate analysis (P < .005 significant). Significant variables were submitted for multivariate analysis. RESULTS: Bronchomalacia was identified in 41% of dogs of all sizes/somatotypes; 38% were >10 kg. All dogs with BM had ≥1 comorbid cardiopulmonary disorder. Dogs with BM were significantly older (P < .001), smaller (P < .001), and were more likely diagnosed with tracheal or mainstem bronchial collapse (P < .001) or bronchiectasis (P < .001). Multivariate analysis confirmed associations with age, tracheal or mainstem bronchial collapse, and bronchiectasis. In dogs with BM, PH was more prevalent. CONCLUSIONS AND CLINICAL IMPORTANCE: Although significantly more common in older, smaller dogs, BM occurs in dogs of all sizes and in all instances with comorbidities. Echocardiography should be considered in dogs with BM to identify PH.

Lower airway collapse: Revisiting the definition and clinicopathologic features of canine bronchomalacia.

Bronchomalacia (BM) is an inconsistently defined term in dogs, impairing understanding of clinical presentation, therapeutic response and prognosis. Herein the authors propose to clarify the definition of canine bronchomalacia (CBM) as regional to diffuse dynamic airway collapse of segmental and/or subsegmental bronchi with associated clinical signs due to airflow limitation. In contrast to tracheal collapse, mainstem bronchial collapse, and in some cases lobar collapse, CBM requires advanced imaging. Common co-morbid conditions (e.g., chronic bronchitis, mitral valve degenerative disease, etc.) should be identified during a comprehensive diagnostic evaluation. Current empiric treatments advocated in absence of clinical trials documenting efficacy in the dog warrant evaluation, as some (e.g., bronchodilators) may have detrimental effects in certain types of airway collapse in humans. There is no direct treatment for CBM, as defined above, but non-specific therapies and targeted treatment of co-morbid disease may improve clinical signs and quality of life. In this manuscript, the authors provide a review of the different types of airway collapse focusing on CBM, reviewing their definition and etiology, proposing a classification scheme, and discussing clinical signs, diagnostic testing, and treatment. Future studies should focus on both improving understanding of the etiology and natural disease progression of CBM and treatment trials.

Bronchial stenting in infants with severe bronchomalacia: Technique and outcomes.

OBJECTIVES: To report the first use of a balloon expandable bare metal stent for treating infant bronchomalacia. BACKGROUND: Infant bronchomalacia often requires prolonged mechanical ventilation and can be life-threatening. Effective treatment for severe infant bronchomalacia continues to be elusive. We present three cases of bronchial stenting for no-option or treatment refractory infant bronchomalacia. METHODS: Three consecutive cases of stenting to relieve conservative treatment refractory severe infant bronchomalacia were performed between February 2019 and December 2020. Initial diagnosis was confirmed with Computed Tomography (CT) angiography. Patients underwent rigid micro laryngoscopy, bronchoscopy, and flexible bronchoscopy to evaluate the airway. Initial conservative management strategies were pursued. Patients failing initial conservative management strategies were considered for rescue bronchial stenting. RESULTS: Our initial clinical experience with a coronary bare metal stent for these procedures has been favorable. The stent was easy to deploy with precision. We did not encounter stent embolization or migration. There was sufficient stent radial strength to relieve bronchomalacia without causing restenosis or erosion. There was no significant granulation tissue formation. In one patient, the stent was removed after 12 months of somatic growth; this was uneventful and bronchial patency was maintained. There were no complications in any of our patients regarding stent placement and reliability. CONCLUSION: In cases of three infants with severe bronchomalacia, we found that bronchial stenting with the bare metal coronary stent was effective in relieving bronchial stenosis.

A Successful Case of External Stenting for Bronchomalacia Lasting Over 20 Years.

We report on a patient with perimembranous ventricular septal defect and pulmonary hypertension. He underwent pulmonary artery banding followed by external stenting of the left bronchus because of secondary bronchomalacia. The external stent applied was a ringed expanded polytetrafluoroethylene graft, 10 mm in diameter and fixed at 8 points. Intracardiac repair was performed at the age of 2 years and the external stent was left in place. Since then, the patient has been healthy and computed tomography reveals that the external stent graft has retained function for over 20 years.

Laryngomalacia, Tracheomalacia and Bronchomalacia.

Airway malacia can occur in the larynx (larygomalacia), trachea (tracheomalacia), or bronchi (bronchomalacia). As a group these are the most common congenital abnormalities of the pediatric airway and are characterized by increased airway compliance, resulting in excessive dynamic collapse during the respiratory cycle. While a diagnosis can be suspected based on clinical history and physical examination, definitive evaluation is based of nasopharyngolaryngoscopy and/or bronchoscopy. Observation and conservative management are typically all that are required. However, surgical intervention can be necessary in the most severe cases, and can result in significant improvement in symptoms.

Successful Infant Pneumonectomy with Unilateral Pulmonary Artery Occlusion Test.

The use of unilateral pulmonary artery occlusion (UPAO) test for the preoperative evaluation of pneumonectomy was reported in adult patients. On the contrary, in infants, no strategies have yet been recommended to predict hemodynamics after pneumonectomy, nor has use of the UPAO test been reported. We describe the first case of infant with abnormal pulmonary circulation in whom successful pneumonectomy was performed after preoperative evaluation using UPAO test. Right pneumonectomy was planned for an 8-month-old girl, because of decreased right pulmonary function, high risk of pneumothorax, and impaired left lung expansion due to overexpansion caused by severe left bronchial stenosis and bronchomalacia. However, she had also prolonged pulmonary hypertension and there was difficulty in accurate echocardiographic evaluation of its severity due to concomitant left pulmonary artery stenosis. Furthermore, contrast-enhanced computer tomography suggested a certain degree of right pulmonary venous flow, discordant with the result showing scarce right pulmonary flow in perfusion scintigraphy. Predicting postoperative hemodynamic changes was therefore considered difficult. To evaluate these concerns, we performed cardiac catheterization and UPAO test to simulate postoperative hemodynamics. Pulmonary arteriography showed decreased but significant right pulmonary arterial and venous flows. Measurements including pulmonary artery pressure and cardiac index showed no marked changes after occlusion. Based on UPAO test results, the operation was successfully performed and hemodynamics remained stable postoperatively. The UPAO test may be useful for infants with cardiopulmonary impairment to evaluate the tolerability of pneumonectomy.

Bronchoscopic assessments and clinical outcomes in pediatric patients with tracheomalacia and bronchomalacia.

BACKGROUND: Tracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM. METHODS: Fifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively. They were divided into two groups according to the presence (group E, n = 26) or absence (group N, n = 31) of acute life-threatening events and extubation failure (ALTE/EF). The severity of TM/BM was evaluated by Oblateness Index which was obtained from bronchoscopic images. RESULTS: Oblateness Index was significantly higher in Group E than in Group N. Patients in Group E underwent surgical intervention for TM/BM more frequently, and had significantly longer intubation period and hospital stay. Clinical symptoms of ALTE/EF, Oblateness Index ≥ 0.70, and multiple malacic lesions were significant risk factors indicating surgical events in patients with TM/BM. CONCLUSIONS: Patients with TM/BM who had ALTE/EF had more severe malacic lesions indicating surgical intervention, and worse clinical outcomes. Oblateness Index is a simple and semi-quantitative index for bronchoscopic assessment of TM/BM, and can be one of the prognostic tools to predict clinical severity of pediatric TM/BM.

Traqueobroncomalacia / Tracheobronchomalacia

A 10-years-old girl with recurrent wheezing was diagnosed as asthmatic. Her spirometry showed variable central airway intrathoracic obstruction. The bronchoscopy confirmed the presence of bronchomalacia.

Se presenta el caso de una paciente de edad escolar, quien por sibilancias recurrentes se trataba como asmática. Al realizar espirometría forzada se detectó un compromiso variable de vía central intratorácica. La fibrobroncoscopía confirmó la presencia de broncomalacia.

Airway complications after single-stage unifocalization for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

OBJECTIVE: We analyze the incidence of postoperative severe airflow limitation after single-stage unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) and comment on the treatment performed. METHODS: From 1994 until 2014, 118 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four patients (3.4%) developed severe airflow complications postoperatively. Chromosome 22q11 deletion was present in three of them. Median age at the time of unifocalization was 6.2 months (range 21 days to 11 months). RESULTS: The first patient developed malacia and compression of the left bronchus from the distal RV-PA conduit and was treated with external bronchial stenting with two incomplete costal cartilage rings. The second patient developed recurrent esophagus-left bronchus fistula treated with multiple surgical esophageal and bronchus reconstructions. The third child presented with bilateral bronchial malacia treated with bilateral stenting followed by surgical elongation of the neo-left pulmonary artery to avoid external compression. The last patient developed bilateral bronchomalacia treated with bilateral bronchial stenting followed by RV-PA conduit replacement and endobronchial stenting calibration. CONCLUSION: Particular categories of patients with PA, VSD, MAPCAs (22q11 chromosome deletion, neonates/infants, patients with dominant/exclusive collaterals) may be more predisposed to develop airway compromise. The treatment of the lesion should be individualized according to the pathogenic mechanism. We suggest endoluminal treatment in absence of compression by vascular structures while surgery was used in case of extrinsic compression.

Complications of long-standing foreign body in the airway and their outcomes after endoscopic management: an experience of 20 cases.

AIM: To study the outcomes after endoscopic treatment of chronic foreign bodies (FBs) in the airway. MATERIALS AND METHODS: A retrospective study (2008-2013) of 20 cases with chronic airway FBs (>2 weeks) was done with emphasis on endoscopic management. All cases were initially evaluated by the pediatric pulmonologist. Flexible and rigid bronchoscopy was done for diagnosis and retrieval, respectively. The techniques of FB retrieval, problems encountered, and their solutions were analyzed. Follow-up flexible bronchoscopy was done in symptomatic cases. Outcomes were assessed in terms of successful removal of the FB, clinical recovery, lung expansion, and need for further procedures. RESULTS: Twenty cases (16 boys, 4 girls) with a mean age of 7 years had a chronic airway FB diagnosed on chest X-ray (n=6) and flexible bronchoscopy (n=14). Six cases had computed tomography evaluation. On rigid bronchoscopy, the FB was successfully retrieved in 16 cases. Two cases required open surgery for FB-induced tracheoesophageal fistula. One case required pneumonectomy because of a battery eroding into the lung parenchyma. One patient died. Of the 16 who had successful retrieval, 11 recovered with full lung expansion. Four recovered after additional bronchoscopic procedures (cauterization of granulation [n=2] and balloon dilatation of bronchial stenoses [n=2]). One case required pneumonectomy for persistent collapse despite multiple dilatations. CONCLUSIONS: An airway FB producing chronic respiratory symptoms may be missed because of lack of definite history of an inhaled FB. Clinical suspicion and flexible bronchoscopy are instrumental in diagnosis. Treatment is challenging because of chronicity-related complications and requires innovative ideas to make best use of the available urologic and bronchoscopic equipment. Addition of tracheotomy provides safety in difficult cases. Bronchoscopic removal leads to clinical and radiological recovery in most cases.

Late-onset Pompe disease with left-sided bronchomalacia.

Pompe disease is a rare autosomal recessive disorder caused by α-glucosidase deficiency. Lower airway involvement and management are rare in patients with late-onset Pompe disease. We describe the case of a 16-y-old girl with late-onset Pompe disease who presented with obvious progressive deterioration in respiratory function. Pulmonary hypertension was also apparent on echocardiography. She had been on enzyme replacement therapy and nighttime CPAP ventilation for several years. Flexible bronchoscopy was used for diagnosis and subsequent implantation of a bronchial airway stent. Following implantation of the stent, the patient's pulmonary function stabilized, and her pulmonary hypertension resolved. The patient continued on enzyme replacement therapy and nighttime CPAP ventilation. This case highlights that lower airway involvement may occur with late-onset Pompe disease and that flexible bronchoscopy can be an effective tool for both diagnosis and management of lower airway collapse in late-onset Pompe disease.

Treatment of bronchus intermedius stenosis in lung transplantation with Montgomery T-tube stent. A novel technique.

Airway complications after lung transplant are relatively common although the rates vary according to the different studies. Pathogenesis is diverse but the principal mechanism is usually bronchus intermedius ischemia in the post-transplant period. One major complication is bronchial stenosis, with relatively frequent involvement of the bronchus intermedius in the case of right lung transplantation. Various treatments have been proposed for bronchus intermedius stenosis, such as endobronchial balloon dilation, laser, cryosurgery and bronchial stents. We present two cases of lung transplant recipients with bronchus intermedius stenosis treated with a Montgomery stent or T-stent, commonly used for tracheal stenosis, who showed positive clinical and functional response.

Bronchial stent placement in a dog with bronchomalacia and left atrial enlargement.

A 13-year-old neutered male Maltese was referred for paroxysms of coughing and cyanosis, with radiographic evidence of bronchial disease and cardiomegaly. Investigation with echocardiography, bronchoscopy, fluoroscopy and bronchoalveolar lavage led to a diagnosis of myxomatous mitral valve degeneration with insufficiency, ISACHC class II heart failure and bronchomalacia with severe left mainstem bronchial collapse. Persistence of intractable cough despite medical therapy prompted placement of a stent in the left mainstem bronchus. Immediately after stent placement, severe pulmonary oedema developed, thought to be due to compression of the left atrium by the stent or acute lung injury related to stent placement. The dog recovered over a 3-day period with diuretic therapy and positive end expiratory pressure ventilation. Subsequently, the dog died from congestive heart failure 102 days after stent placement, during which time occasional, self limiting coughing episodes occurred.

Isolated severe bilateral bronchomalacia.

Airway malacia is uncommon condition having symptoms similar to common respiratory illnesses. Any child having persistent wheeze during infancy should be evaluated for airway malacia. The authors report a case of isolated severe bilateral bronchomalacia managed with tracheostomy and continuous positive pressure ventilation.