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.

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.

Successful biodegradable stent insertion in an infant with severe bronchomalacia and cystic fibrosis.

We report the first case of biodegradable airway stent insertion for a patient with bronchomalacia and cystic fibrosis (CF). This female infant with antenatally diagnosed cystic fibrosis developed respiratory distress by three weeks of age. On lower airway examination she was found to have severe left main stem bronchomalacia causing left upper lobe hyperinflation and subsequent right upper lobe collapse. By eight weeks of age she developed life-threatening respiratory failure requiring high pressure invasive ventilation. A biodegradable bronchial stent was inserted to the left main bronchus at thirteen weeks of age with successful extubation from invasive respiratory support four days later. A second biodegradable stent was inserted nine weeks later, due to persistent bronchomalacia following stent degradation and redevelopment of oxygen requirement. She was discharged home off all respiratory support eight weeks later and has remained well, requiring no further surgical intervention for bronchomalacia to date, now age three years.

ERS statement on tracheomalacia and bronchomalacia in children.

Tracheomalacia and tracheobronchomalacia may be primary abnormalities of the large airways or associated with a wide variety of congenital and acquired conditions. The evidence on diagnosis, classification and management is scant. There is no universally accepted classification of severity. Clinical presentation includes early-onset stridor or fixed wheeze, recurrent infections, brassy cough and even near-death attacks, depending on the site and severity of the lesion. Diagnosis is usually made by flexible bronchoscopy in a free-breathing child but may also be shown by other dynamic imaging techniques such as low-contrast volume bronchography, computed tomography or magnetic resonance imaging. Lung function testing can provide supportive evidence but is not diagnostic. Management may be medical or surgical, depending on the nature and severity of the lesions, but the evidence base for any therapy is limited. While medical options that include bronchodilators, anti-muscarinic agents, mucolytics and antibiotics (as well as treatment of comorbidities and associated conditions) are used, there is currently little evidence for benefit. Chest physiotherapy is commonly prescribed, but the evidence base is poor. When symptoms are severe, surgical options include aortopexy or posterior tracheopexy, tracheal resection of short affected segments, internal stents and external airway splinting. If respiratory support is needed, continuous positive airway pressure is the most commonly used modality either via a face mask or tracheostomy. Parents of children with tracheobronchomalacia report diagnostic delays and anxieties about how to manage their child's condition, and want more information. There is a need for more research to establish an evidence base for malacia. This European Respiratory Society statement provides a review of the current literature to inform future study.

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.

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.

Post-pneumonectomy and post-lobectomy syndromes: case series and review of the literature.

The post-pneumonectomy syndrome is a rare complication consisting of rotation and herniation of the mediastinal structures, the remaining lung and the respective bronchi, into the contralateral hemithorax. This situation may produce symptomatic airway obstruction and varies in its presentation and severity. We describe one case of right and one of left pneumonectomy syndrome as well as one case of post-lobectomy syndrome. We review the literature on the pathophysiology, the clinical, radiological and bronchoscopic characteristics of this rare entity and discuss all available therapeutic alternatives.

Biodegradable airway stents in infants - Potential life-threatening pitfalls.

OBJECTIVE: The solution of severe tracheobronchial obstructions in early childhood means a great challenge. Biodegradable stents were intended to be a minimally invasive temporary solution which may decrease the number of interventions and limit the possible complications of stenting procedures. However, our first experiences have brought out a new, - especially in childhood - potentially life-threatening complication of this concept. METHODS: Five SX-ELLA biodegradable polydioxanone stents was applied in three patients because of severe tracheobronchial obstruction: congenital tracheomalacia (7 day-old), acquired tracheomalacia (10 month-old), and congenital trachea-bronchomalacia (10 month-old). RESULTS: The breathing of all children improved right after the procedure. We observed degradation of the stent from the 5th postoperative week which resulted in large intraluminar fragments causing significant airway obstruction: one patient died of severe pneumonia, the other baby required urgent bronchoscopy to remove the obstructing 'foreign body' from the trachea. In the third case repeated stent placements successfully maintained the tracheal lumen. CONCLUSIONS: Polydioxanone stents may offer an alternative to metallic or silastic stents for collapse or external compression of the trachea in children; however, large decaying fragments mean a potential risk especially in the small size pediatric airway. The fragmentation of the stent, which generally starts in the 4-6 postoperative weeks, may create large sharp pieces. These may be anchored to the mucosa and covered by crust leading to obstruction. As repeated interventions are required, we do not consider the application of biodegradable stents unambiguously advantageous.

Transplant Pulmonary Interventions: Translating Lung Transplant Interventions to Nontransplant Patients.

Roughly 10% of lung transplant recipients experience airway complications. Although the incidence has decreased dramatically since the first lung transplants were performed in the 1960s, airway complications have continued to adversely affect outcomes. Bronchoscopic interventions such as balloon dilation, airway stenting, and endobronchial electrocautery play an important role in ameliorating the morbidity and mortality associated with these complications. This review describes the array of bronchoscopic interventions used to treat airway complications after lung transplant and how these techniques can be used in nontransplant settings as well.

Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia.

Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.

Airway Disease and Management in Bronchopulmonary Dysplasia.

This article presents an overview of the diagnosis and management of airway problems encountered in infants with severe bronchopulmonary dysplasia (BPD). Respiratory failure in premature infants develops as a result of parenchymal and airway diseases. The survival of increasingly premature infants and the ventilatory support required by premature lungs may result in airway disease. The management of respiratory failure depends on whether it is primarily caused by parenchymal versus airway diseases. Continuous airway pressure early in the neonatal period has favorably changed the incidence of BPD. This article discusses the indications, timing, and guidelines for care of tracheotomy.

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.

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.

Tracheal and airway collapse in dogs.

Tracheal and airway collapse (bronchomalacia) are common causes of chronic cough in middle-aged to older dogs where weakening of cartilage within the respiratory system leads to narrowing of airways, coughing, wheezing, and other secondary effects. Successful treatment involves correct identification of the problem, recognition of concurrent problems, and appropriate medical therapy. Surgical and noninvasive treatment options are becoming readily available, and it is important to understand indications for such procedures.

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.

Left pulmonary artery sling with right lung aplasia.

We report 2 cases of right pulmonary aplasia with left pulmonary artery sling responsible for severe respiratory symptoms. Repositioning of the left pulmonary artery without tracheal surgery was successful in both patients. The postoperative course was simple, and the outcome was favorable at last follow-up (after 2 years and 3 months, respectively). Computed tomography provided an accurate diagnostic evaluation that helped to choose the best surgical technique.

Respiratory failure of Williams-Campbell syndrome is effectively treated by noninvasive positive pressure ventilation.

Williams-Campbell syndrome is a rare disease, characterized by a congenital deficiency of cartilage in the fourth to sixth order bronchi, leading to chronic respiratory failure with recurrent pulmonary infections. An effective and practical treatment has not yet been established. A 31-year-old man who was diagnosed as Williams-Campbell syndrome by inspiratory and expiratory computed tomography findings developed recurrent pulmonary infections and showed progressive deterioration of dyspnea. Domiciliary NPPV was administered, followed by a dramatic improvement of respiratory failure and a decrease in the episodes of pulmonary infections. NPPV may have an advantage in adults with Williams-Campbell syndrome who have severe respiratory failure and recurrent pulmonary infections.

Bronchomegaly as a complication of fetal endoscopic tracheal occlusion. A caution and a possible solution.

Fetal medicine is developing rapidly and aims to improve the outcome for fetuses with congenital anomalies. Fetal endoscopic tracheal occlusion (FETO) has been developed for fetuses with congenital diaphragmatic hernia to counterbalance the compression of the lung by the abdominal viscera, preserving the pulmonary maturation. Because the perinatal morbidity and mortality of patients treated with FETO have decreased, new complications are emerging in the older survivors. Tracheomegaly has been reported to be a late complication of FETO, sometimes requiring tracheostomy. We report a case of bronchial dilatation after FETO and suggest an alternative surgical treatment.

Treatment of airway complications following lung transplantation.

OBJECTIVE: To describe our experience in airway complications following lung transplant and to suggest a management algorithm, using different tools from the Interventional Pulmonology armamentarium. METHOD: Retrospective chart review of all airway complications following lung transplant from January 1999 to July 2007. RESULTS: During that period 223 patients underwent lung transplantation, with a total of 345 anastomoses in the airway. Seventy anastomoses (20.23%) had complications requiring endoscopic treatment. The total number of endoscopic interventions were 631 in 52 patients. Thirty three patients had a combination of bronchial stenosis and bronchomalacia. Eighteen patients had bronchial stenosis only and 1 patient had dehiscence of the anastomosis. Balloon dilation was most commonly transiently effective and ultimately 47 patients required stent placement. The most common complication associated with the use of stent was granulation tissue formation, seen in 57.3% of patients. After stent placement, the forced expiratory volume in 1(st) second (FEV(1)) improved significantly. CONCLUSION: Airway complications after lung transplant are frequent. Balloon dilation was effective only in a few patients with bronchial stenosis, although the majority ultimately needed a stent. Airway repermeabilization after stent placement improved FEV(1). Based on our experience, we propose a management algorithm for airway complications after lung transplant.