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Orphan lung diseases
S56 Flat Trachea Syndrome: an under-diagnosed and under-treated condition?
  1. G Niranjan,
  2. J F K Marzouk
  1. University Hospital of Coventry and Warwickshire, Coventry, UK

Abstract

Objectives Flat Trachea Syndrome (Tracheobronchomalacia) is a central airway disease characterised by weakness of the wall and dynamic decrease in the tracheal lumen and the large bronchi, particularly while exhaling. It causes chronic symptoms such as cough, dyspnoea, increase in recurrent infections, and poor secretion management, but it can also progress to chronic respiratory failure and death. It remains largely under-diagnosed unless clinicians are thoroughly acquainted with its peculiar symptomology being usually confused with other common diseases like chronic obstructive pulmonary disease (COPD) or asthma. Surgery with posterior tracheobronchial splinting (tracheobronchoplasty) using a polytetrafluoroethylene (PTFE) Teflon patch has been considered as a possible treatment option for this condition.

Methods A retrospective series of 28 patients that underwent tracheobronchoplasty using a PTFE Teflon patch between July 1998 and July 2010 were evaluated for symptoms, lung capacity and exercise capacity.

Results The age range of patients treated was from 24 to 82 (mean 66). 61% (17) of those treated were men, with all cases being diagnosed with obstructive airways disease on spirometry, with the FEV1 ranging from 34% to 65% pre-operatively. 25 patients (89%) presented with severe dyspnoea, 23 patients (82%) with uncontrollable cough, and 21 patients (75%) reported recurrent pulmonary infections. There was no in-hospital or 30-day mortality. After surgery symptomatic improvement in dyspnoea was reported in 24 of 28 patients (p<0.001), with the cough disappearing in 19 of 21 patients (p<0.001). At 3 months the FEV1 was >80% in all 28 patients. The mean exercise capacity was improved in 17 (61%) patients.

Conclusions The condition of tracheobronchomalacia is a misnomer and we propose the term “Flat Trachea syndrome” and/or “expiratory prolapse of the tracheobronchial posterior membrane”. It is a rare but severely debilitating condition which can be diagnosed easily by an awake flexible bronchoscopy and dynamic biphasic inspiratory/expiratory CT. Surgical airway splinting with a PTFE (Teflon) patch considerably improves respiratory symptoms, quality of life and functional status in highly selected patients with this under-diagnosed and under-treated condition.

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