An 18-year-old patient with metabolic myopathy due to long-chain fatty acid coenzyme A dehydrogenase-deficiency1 ,2 presented with worsening positional dyspnoea. He had required invasive ventilation via tracheostomy 6 months earlier. Examination revealed respiratory distress, a prolonged inspiratory phase and abdominal paradox. Non-invasive ventilation improved dyspnoea but surprisingly high inspiratory pressures were required. CT imaging revealed subtle, sublaryngeal irregularity. Volumetric reconstruction identified a thin tracheal web (figure 1). Diathermy via rigid bronchoscopy (figure 2) immediately improved ventilation. Our patient had the potentially deadly combination of a pinhole trachea with respiratory muscle weakness, unable to generate audible stridor. Clinicians should be aware of limitations in conventional CT assessment of the upper airway.
Publication information
Nanzer AM, Jordan S, Paddley Set al 'A deadly web. Case Report' Thorax. 2015 Jan;70(1):101