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Upper airway bronchoscopic interpretation
Published in Don Hayes, Kara D. Meister, Pediatric Bronchoscopy for Clinicians, 2023
Anita Deshpande, Cherie A. Torres-Silva, Catherine K. Hart
Airway malacia, which includes laryngomalacia, tracheomalacia, and bronchomalacia, is a dynamic obstruction of the airways and is the most common congenital airway anomaly in children. Laryngomalacia is a condition in which supraglottic structures, including the aryepiglottic folds, the arytenoid, corniculate, or cuneiform cartilages, the epiglottis, or a combination of all the above, collapse into the larynx and obstruct airflow during inspiration (Video 4.1). Flexible bronchoscopy via a trans-nasal approach during spontaneous ventilation allows for thorough dynamic evaluation. Common findings include shortened aryepiglottic folds, an omega-shaped epiglottis, and redundant arytenoid mucosa that can prolapse into the larynx.17
Congenital airway malformations
Published in Prem Puri, Newborn Surgery, 2017
Alessandro de Alarcón, Richard G. Azizkhan
Airway malacia is a condition in which the structural integrity of either the trachea or bronchi or both are weakened, and the cartilaginous rings of the airway lack the rigidity required to avoid airway collapse during expiration. Malacia may be localized or occur diffusely throughout the airway. Tracheomalacia is the most common congenital tracheal anomaly. This condition may occur in isolation or in conjunction with other congenital anomalies. TEF, esophageal atresia, and posterior laryngeal clefts are particularly common associations.54 Premature neonates and children with chronic lung disease are at high risk for developing combined severe tracheobronchomalacia.
Diagnosis of Asthma in Preschool-Age Children
Published in Jonathan A. Bernstein, Mark L. Levy, Clinical Asthma, 2014
Chris Cleveland, Lora Stewart, Bradley Chipps, Joseph D. Spahn
Tracheomalacia is a common anatomical defect seen in young children. Patients with tracheomalacia often present with recurrent episodes of stridor, wheeze, or barky cough, which are worsened by crying, and a concurrent respiratory infection.49,50 The respiratory symptoms often subside while sleeping, in contrast to asthma symptoms that often worsen at night. In many patients, the symptoms are present between episodes of infection, but mild cases may only be symptomatic with infection or vigorous crying. Additionally, treatment with a bronchodilator results in no change or even worsens the symptoms because it results in diminished airway tone in the presence of malacia. Glucocorticoids, both systemic and inhaled, are also ineffective. For the majority of patients, the symptoms associated with tracheomalacia resolve by age 2, but more persistent disease can occur. An evaluation for less common etiologies, including a tracheoesophageal fistula (TEF), a vascular ring, or an underlying connective tissue disorder, should be pursued for severe or persistent presentations. A bronchoscopy is the examination of choice in the diagnosis and assessment of tracheomalacia.
The role of the pediatrician in caring for children with tracheobronchomalacia
Published in Expert Review of Respiratory Medicine, 2020
Manisha Ramphul, Andrew Bush, Anne Chang, Kostas N Prifits, Colin Wallis, Jayesh Mahendra Bhatt
Interestingly, the etymology of malacia originates from the Greek word ’malakia’, meaning soft [6]. Laryngomalacia, a congenital softening of the tissues of the larynx above the vocal cords, is commonly encountered in pediatric practice as a cause of noisy breathing and will not be reviewed in this paper.
Mounier Kuhn syndrome presenting with recurrent atelectasis
Published in Acta Clinica Belgica, 2018
Christine Quentin, Nicolas Lefevre, Eddy Bodart, Laurence Hanssens
Mounier Kuhn syndrome is characterized by abnormal enlargement of the major airways. The primary respiratory symptoms are usually non-specific such as cough, dyspnea, or recurrent respiratory infections [5]. Radiologic features might be seen on chest X-rays but are very often overlooked as described in our case. Since 1988, chest CT is the gold standard to confirm the diagnosis [7]. Magnetic resonance imaging does not have any supplementary advantage in diagnosis. Bronchoscopy can be difficult to perform because of important airway obstruction due to tracheomalacia. Spirometry can show different degrees of obstruction or increased residual function. Differential diagnosis should consider William Campbell syndrome, Ehlers-Danlos syndrome, or Marfan syndrome. Most frequent pulmonary complications are bullous emphysema, aspergillosis, and pneumothorax. Management is based on supportive treatment with physiotherapy and inhaled mucolytic therapy [5]. Pneumococcal and seasonal influenza vaccination is useful. Appropriate antibiotic therapy in case of pulmonary exacerbation might be guided by guidelines of non-cystic fibrosis bronchiectasis [8]. Non-invasive ventilator support can help to cope with the malacia. Surgical treatment of the tracheomalacia by tracheobronchoplasty or the use of airway stents has been described. Cases of Mounier Kuhn syndrome with a wide variety of clinical presentations and treatment options have been reported – including patients with P. aeruginosa. [9] However, in our case, the patient did not show any bronchiectasis. Impaired airway clearance in Mounier Kuhn syndrome caused by expiratory airway-collapse due to tracheobronchomegaly in association with chronic inflammation might promote this colonization. As no specific guidelines for the treatment of pulmonary exacerbation in Mounier Kuhn syndrome exist, we decided to treat our patient with oral antibiotic therapy according to guidelines of P. aeruginosa management in cystic fibrosis patients because of its similar pathophysiology. We were not able to add inhaled antibiotics because of its high cost and lack of mutual refund as recommended by P. aeruginosa guidelines [10,11]. Our case highlights the value of standard chest X-ray which can still offer valuable information in many diseases and, in some cases, avoid numerous and unnecessary complicated investigations. This case report of a rare disease also gives us the opportunity to share our experience of treating a patient with Mounier Kuhn syndrome and in particular a pulmonary exacerbation caused by P. aeruginosa.