Presentation, diagnosis and management of congenital long-segment tracheal stenosis: a single-centre experience

Abstract

Congenital long-segment tracheal stenosis (CLSTS) is a rare airway malformation, caused by absence of the pars membranacea in part of the trachea. 

The aim of this study is to provide our single-centre experience with CLSTS and a literature review on clinical presentation, diagnostic workup, treatment methods and outcome. Special attention will be given to the relatively new technique for anatomic visualisation, using anatomic optical coherence tomography (aOCT).

Methods: Single-centre retrospective cohort study of children diagnosed with CLSTS and treated at the University Hospital of Leuven between January 2010 and July 2021. A literature review was performed using PubMed.

Results: Six children were included, age at diagnosis between 11 days and 12 years. Three children had associated pulmonary artery sling and one had unilateral agenesis of the lung, both requiring additional surgery. Presenting symptoms were mainly stridor and respiratory insufficiency during lower airway infections. Diagnosis was made by a combination of bronchoscopy, chest CT, bronchography and aOCT. Four patients have been treated surgically and one is awaiting surgery; one was treated conservatively. One patient suffered from important cardiac ischemia postoperatively. Three patients required balloon dilation and one required additional pericardial patch tracheoplasty. Follow-up after diagnosis ranged between two and eight years. One patient still has stridor with exercise, two still have intermittent stridor with respiratory tract infections after surgery.

Interpretation: Outcome in our study population was consistent with data from the literature. CLSTS requires a dedicated and multidisciplinary approach to obtain optimal results. aOCT has proven its added value for diagnosis.