Background: Tracheomalacia (TM) and tracheobronchomalacia (TBM) are conditions of excessive tracheal collapsibility, due either to disproportionate laxity of the posterior wall (pars membranacea) or compromised cartilage integrity. As a result, the anterior and posterior walls oppose, reducing the tracheal lumen opening and creating a shape abnormality during bronchoscopy. Despite the frequency of TBM seen by paediatric respiratory specialists, there are many current controversies.
Objective: A Task Force with European Respiratory Society was established to review the current literature in children describing the evidence for diagnosis, clinical impact and therapeutic options, and the impact on families and patients with suggestions for future research.
Results: 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 (low contrast volume bronchography, CT or MRI. 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 are used, there is currently little evidence for benefit. When symptoms are severe, surgical options include anterior or posterior tracheopexy, tracheal resection of short affected segments, internal stents and external airway splinting. If respiratory support is needed, CPAP is the most commonly used modality either via a facial mask or tracheostomy. Parents of children with TBM report diagnostic delays and anxieties about their child’s condition and want more information.
Conclusion: There is a need for more research to establish an evidence base for malacia.