Tracheocoele in a Duchenne muscular dystrophy patient. Case report

Tracheocoele, a congenital or acquired lesion, is rarely detected radiologically and even more rarely diagnosed clinically. This tracheal lesion is characterised by the presence of a single cystic lesion filled with air or a mixture of liquid and air, of extremely variable size, occurring in almost all cases, in a locus minoris resistentiae situated in the right posterolateral portion of the trachea. The rare case is described of a voluminous tracheocoele located in the left paratracheal region, extending from the cricoid to sternal notch, manifesting clinically, 3 months prior to evaluation in our hospital in a 27-year-old male suffering from Duchenne muscular dystrophy since the age of 5 years. For 10 years, the patient had been treated with intermittent positive pressure ventilation via nasal mask, due to progressive deterioration of respiratory function. Diagnosis of tracheocoele, initially made at computed axial tomography scan, was confirmed by flexible laryngotracheoscopy under local anaesthesia. Due to severe comorbidity associated with the clinical picture described, the absence of a significant set of symptoms, and the problems concerning anaesthesiological management of the patient, palliative treatment was the only choice. This consisted in cervical compression bandaging during assisted nasal ventilation. Close follow-up was performed in order to monitor any progression of the lesion or onset of related complications. This is the second case of tracheocoele originating in the left paratracheal region reported in the literature, and the first associated with Duchenne muscular dystrophy and prolonged use of a positive pressure respirator. The aetiopathogenic mechanisms that may have determined the formation of this rare lesion are then taken into consideration.