ERS statement on tracheomalacia and bronchomalacia in children
Colin Wallis, Efthymia Alexopoulou, Juan L Antón-Pacheco, Jayesh M Bhatt, Andrew Bush, Anne B Chang, Anne-Marie Charatsi, Courtney Coleman, Julie Depiazzi, Konstantinos Douros, Ernst Eber, Mark Everard, Ahmed Kantar, Ian B Masters, Fabio Midulla, Raffaella Nenna, Derek Roebuck, Deborah Snijders, Kostas Priftis, Colin Wallis, Efthymia Alexopoulou, Juan L Antón-Pacheco, Jayesh M Bhatt, Andrew Bush, Anne B Chang, Anne-Marie Charatsi, Courtney Coleman, Julie Depiazzi, Konstantinos Douros, Ernst Eber, Mark Everard, Ahmed Kantar, Ian B Masters, Fabio Midulla, Raffaella Nenna, Derek Roebuck, Deborah Snijders, Kostas Priftis
Abstract
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.
Conflict of interest statement
Conflict of interest: C. Wallis has nothing to disclose. Conflict of interest: E. Alexopoulou has nothing to disclose. Conflict of interest: J.L. Antón-Pacheco has nothing to disclose. Conflict of interest: J.M. Bhatt reports personal fees from Vertex, outside the submitted work. Conflict of interest: A. Bush has nothing to disclose. Conflict of interest: A.B. Chang reports grants from National Health and Medical Research Council, Australia, and other funding from GSK, Up to Date and BMJ Evidence Centre, outside the submitted work. Conflict of interest: A-M. Charatsi has nothing to disclose. Conflict of interest: C. Coleman is an employee of European Lung Foundation. Conflict of interest: J. Depiazzi has nothing to disclose. Conflict of interest: K. Douros has nothing to disclose. Conflict of interest: E. Eber has nothing to disclose. Conflict of interest: M. Everard has nothing to disclose. Conflict of interest: A. Kantar has nothing to disclose. Conflict of interest: I.B. Masters has nothing to disclose. Conflict of interest: F. Midulla has nothing to disclose. Conflict of interest: R. Nenna has nothing to disclose. Conflict of interest: D. Roebuck has nothing to disclose. Conflict of interest: D. Snijders has nothing to disclose. Conflict of interest: K. Priftis has nothing to disclose.
Copyright ©ERS 2019.
Source: PubMed