Dynamic computed tomography for evaluation of tracheobronchomalacia in premature infants with bronchopulmonary dysplasia.

INTRODUCTION: Dynamic computed tomography (dCT) gives real-time physiological information and objective descriptions of airway narrowing in tracheobronchomalacia (TBM). There is a paucity of literature in the evaluation of TBM by dCT in premature infants with bronchopulmonary dysplasia (BPD). The aim of this study is to describe the findings of dCT and resultant changes in management in premature infants with TBM. METHODS: A retrospective study of 70 infants was performed. Infants included were <32 weeks gestation without major anomalies. TBM was defined as ≥50% expiratory reduction in cross-sectional area with severity defined as mild (50%-75%), moderate (≥75%-90%), or severe (≥90%). RESULTS: Dynamic CT diagnosed malacia in 53% of infants. Tracheomalacia was identified in 49% of infants with severity as 76% mild, 18% moderate, and 6% severe. Bronchomalacia was identified in 43% of infants with varying severity (53% mild, 40% moderate, 7% severe). Resultant management changes included PEEP titration (44%), initiation of bethanechol (23%), planned tracheostomy (20%), extubation trial (13%), and inhaled ipratropium bromide (7%). CONCLUSION: Dynamic CT is a useful noninvasive diagnostic tool for airway evaluation of premature infants. Presence and severity of TBM can provide actionable information to guide more precise clinical decision making.

Tracheopexy and aortopexy for circumflex aortic arch on a small infant.

Circumflex aortic arch is a rare vascular ring anomaly, which is often associated with tracheobronchomalacia. Aortic uncrossing is the definitive repair of this condition, which is a major high-risk operation; therefore, it is not suitable for small patients such as newborn infants or individuals who have severe comorbidities. We present a case of the circumflex aortic arch with tracheobronchomalacia in a 2.7-kg infant who underwent successful innovative combined surgery of the airway and the aorta.

Tracheobronchomalacia and Excessive Dynamic Airway Collapse: Current Concepts and Future Directions.

Tracheobronchomalacia (TBM) and excessive dynamic airway collapse (EDAC) are airway abnormalities that share a common feature of expiratory narrowing but are distinct pathophysiologic entities. Both entities are collectively referred to as expiratory central airway collapse (ECAC). The malacia or weakness of cartilage that supports the tracheobronchial tree may occur only in the trachea (ie, tracheomalacia), in both the trachea and bronchi (TBM), or only in the bronchi (bronchomalacia). On the other hand, EDAC refers to excessive anterior bowing of the posterior membrane into the airway lumen with intact cartilage. Clinical diagnosis is often confounded by comorbidities including asthma, chronic obstructive pulmonary disease, obesity, hypoventilation syndrome, and gastroesophageal reflux disease. Additional challenges include the underrecognition of ECAC at imaging; the interchangeable use of the terms TBM and EDAC in the literature, which leads to confusion; and the lack of clear guidelines for diagnosis and treatment. The use of CT is growing for evaluation of the morphology of the airway, tracheobronchial collapsibility, and extrinsic disease processes that can narrow the trachea. MRI is an alternative tool, although it is not as widely available and is not used as frequently for this indication as is CT. Together, these tools not only enable diagnosis, but also provide a road map to clinicians and surgeons for planning treatment. In addition, CT datasets can be used for 3D printing of personalized medical devices such as stents and splints. An invited commentary by Brixey is available online. Online supplemental material is available for this article. ©RSNA, 2022.

Dynamic PEEP Study: A Non-invasive Diagnostic Exam to Assess for Effective PEEP in Children with Severe BPD.

OBJECTIVE: Tracheobronchomalacia (TBM) is common in neonates with bronchopulmonary dysplasia (BPD) and is associated with higher morbidity. This study evaluates the value of a CT protocol to assess the degree of TBM and gauge the adequacy of prescribed PEEP. STUDY DESIGN: Four infants with severe BPD on invasive mechanical ventilation underwent a chest CT protocol, including limited reduced-dose expiratory scans with varying PEEP levels. RESULTS: Baseline PEEP was adjusted in all subjects after performing the Dynamic PEEP CT. In two infants, the PEEP was increased due to significant TBM and in the other two without signs of TBM PEEP was decreased. The clinical course improved in all patients after adjusting PEEP. CONCLUSION: A "Dynamic PEEP" study may be reliable and non-invasive imaging modality for the evaluation of adequate ventilator settings in infants with severe BPD who are not optimal candidates for bronchoscopy.

A 70-Year-Old Man With Cough and Recurrent Respiratory Infections.

CASE PRESENTATION: A 70-year-old man was referred for evaluation of recurrent respiratory infections requiring antibiotics and chronic cough over 3 years. Two months prior to presentation, he started to develop blood-tinged sputum but not frank hemoptysis. He otherwise denied any fever, chills, night sweats, or weight loss. He had dyspnea during the respiratory infections but not otherwise. His medical history was significant for chronic rhinitis without sinusitis and a low serum IgM level. He was a never smoker and a farmer but otherwise had no significant or specific exposures or travel history. His family history was significant for alpha-1 antitrypsin deficiency in his mother.

A modified surgical technique for aortopexy in tracheobronchomalacia.

OBJECTIVES: Tracheobronchomalacia (TBM) is characterized by collapse of trachea, bronchi or both, leading to dyspnoea, expiratory stridor, coughing or recurrent airway infections. Surgical treatment with aortopexy is warranted for severe TBM. We describe a modified aortopexy technique with aortic wall strap sutures that evenly distributes the traction force over the full width of the aortic arch. The aim of this study was to determine the outcomes of this modified anterior aortopexy technique. METHODS: Retrospective chart review of all patients undergoing aortopexy with aortic wall strap sutures for TBM between January 2010 and June 2020 in 2 tertiary hospitals in the Netherlands. RESULTS: Twenty-four patients [median age 9 months (interquartile range 2-117 months); 71% male] underwent aortopexy with the modified technique for TBM (52%), tracheomalacia (40%) or bonchomalacia (8%). Aortopexy was successful in 91.7%, defined as relief or decrease of respiratory symptoms and no need for respiratory support. Complications occurred in 8.3% and mortality was 4%. CONCLUSIONS: Aortopexy with non-absorbable strap sutures seems an effective and safe treatment for severe TBM. This study supports the hypothesis that strap sutures provide a solid and reliable traction force, but future comparative studies should confirm the benefit of strap sutures over conventional techniques.

Aortic uncrossing and tracheobronchopexy corrects tracheal compression and tracheobronchomalacia associated with circumflex aortic arch.

OBJECTIVE: Aortic uncrossing is an effective procedure for relieving the external airway compression from a circumflex aortic arch by transferring the aortic arch to the same side as the descending aorta. However, patients frequently have residual tracheobronchomalacia (TBM), which may result in persistent postoperative symptoms. We review a series of patients who underwent an aortic uncrossing and concomitant tracheobronchopexy to correct the airway compression and residual TBM. METHODS: Retrospective review of all patients who underwent aortic uncrossing and concomitant tracheobronchopexy at a single institution between September 2016 and March 2019. Preoperative evaluation included computed tomography angiography and rigid 3-phase dynamic bronchoscopy. RESULTS: Eight patients who ranged in age from 4 months to 15 years with significant respiratory symptoms underwent an aortic uncrossing procedure with concomitant tracheobronchial procedures. Mild hypothermic cardiopulmonary bypass (mean time, 105.6 ± 39.4 min) and regional perfusion (mean time, 44 ± 10 min) were used without circulatory arrest. Intraoperative bronchoscopy demonstrated no patients had residual TBM. There were no postoperative mortalities, neurologic complications, chylothoraces, coarctations, or obstructed aortic arches. Two patients required tracheostomy and gastrostomy for bilateral recurrent laryngeal nerve paresis (patients 2 and 3). One patient with bronchial stenosis after concomitant slide bronchoplasty required stenting. At a median follow-up of 22 months (range, 5-34 months), all patients were alive without evidence of significant respiratory symptoms. CONCLUSIONS: The aortic uncrossing procedure can be performed safely in pediatric patients of all ages without circulatory arrest. Concomitant procedures addressing associated TBM can significantly improve respiratory symptoms.

Williams-Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report.

Williams-Campbell syndrome, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis, which typically affects the fourth- to sixth-order bronchi. This article reported a 31-year-old female patient who was diagnosed with Williams-Campbell syndrome with pulmonary hypertension and Type 2 respiratory failure due to extensive cystic bronchiectasis. CT of the thorax showed the affected bronchi had characteristic ballooning on inspiration and collapse on expiration.

Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis.

OBJECTIVES: To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease. METHODS: Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A subset of patients with GPA from a single-centre cohort underwent dynamic chest CT to evaluate the airways. RESULTS: Among 962 patients with GPA, SGS and endobronchial disease were identified in 95 (10%) and 59 (6%) patients, respectively. Patients with SGS were more likely to be female (72% vs 53%, P < 0.01), younger at time of diagnosis (36 vs 49 years, P < 0.01), and have saddle-nose deformities (28% vs 10%, P < 0.01), but were less likely to have renal involvement (39% vs 62%, P < 0.01). Patients with endobronchial disease were more likely to be PR3-ANCA positive (85% vs 66%, P < 0.01), with more ENT involvement (97% vs 77%, P < 0.01) and less renal involvement (42% vs 62%, P < 0.01). Disease activity in patients with large-airway disease was commonly isolated to the subglottis/upper airway (57%) or bronchi (32%). Seven of 23 patients screened by dynamic chest CT had large-airway pathology, including four patients with chronic, unexplained cough, discovered to have tracheobronchomalacia. CONCLUSION: SGS and endobronchial disease occur in 10% and 6% of patients with GPA, respectively, and may occur without disease activity in other organs. Dynamic expiratory chest CT is a potential non-invasive screening test for large-airway involvement in GPA.

Biodegradable airway stents: Novel treatment of airway obstruction in children.

BACKGROUND: Tracheobronchomalacia is the most common cause of congenital airway obstruction in infants. An alternative for surgical approach (aortopexy) can be metallic airway stents. Usually, they are not considered as a first choice because of the relatively high risk of complications. Recent years have brought encouraging reports of biodegradable stents applied in children. OBJECTIVES: The aim of this study was to report our experience in the treatment of airway malacia using biodegradable stents. MATERIAL AND METHODS: Six polydioxanone (PDS), self-expanding custom-made stents (ELLA-CS) were implanted in 2 children: 3 in the patient with left main bronchus occlusion due to postpnemonectomy syndrome and 3 stents in the baby with tracheomalacia. RESULTS: Airway collapse was always relieved after stent expansion. Both patients needed repeated stenting because of limited stent lifespan. All the stents were implanted without complications through a rigid bronchoscope. The baby with stented main bronchus died because of irreversible lung lesion. CONCLUSIONS: This small study shows that biodegradable airway stents seem to be an attractive option in the treatment of tracheobronchomalacia in children. We consider this method to be safe, effective, repeatable, and reversible in small children with growing airways. As a time-buying procedure they can be especially useful in the treatment of tracheobronchomalacia.

An Adult Case of Pulmonary Artery Sling Accompanied by Tracheobronchomalacia.

Pulmonary artery (PA) sling is a congenital disease in which the left PA abnormally arises from the right PA and is usually diagnosed during the infantile period. We present an adult case of PA sling accompanied by tracheobronchomalacia found in a 49-year-old woman with a history of recurrent pneumonia. Computed tomography of the chest showed that the left lung was nourished by two aberrant PAs. Bronchoscopy demonstrated achondroplasia of the trachea and the right bronchus, which we speculate to have resulted in their stenosis. The recurrent pneumonia was attributable to these tracheobronchial structural abnormalities; we therefore stress the importance of focusing on the anatomic abnormalities in such cases.

Technique, pitfalls, quality, radiation dose and findings of dynamic 4-dimensional computed tomography for airway imaging in infants and children.

This retrospective review of 33 children's dynamic 4-dimensional (4-D) computed tomography (CT) images of the airways, performed using volume scanning on a 320-detector array without anaesthesia (free-breathing) and 1.4-s continuous scanning, was undertaken to report technique, pitfalls, quality, radiation doses and findings. Tracheobronchomalacia (airway diameter collapse >28%) was recorded. Age-matched routine chest CT scans and bronchograms acted as benchmarks for comparing effective dose. Pitfalls included failure to administer intravenous contrast, pull back endotracheal tubes and/or remove nasogastric tubes. Twenty-two studies (67%) were diagnostic. Motion artefact was present in 16 (48%). Mean effective dose: dynamic 4-D CT 1.0 mSv; routine CT chest, 1.0 mSv, and bronchograms, 1.4 mSv. Dynamic 4-D CT showed tracheobronchomalacia in 20 patients (61%) and cardiovascular abnormalities in 12 (36%). Fourteen children (70%) with tracheobronchomalacia were managed successfully by optimising conservative management, 5 (25%) underwent surgical interventions and 1 (5%) died from the presenting disorder.