METHODS: A 22-year-old male patient with DMD presented at emergency department due dyspnoea starting at 3 days associated with right scapular
pain, enhanced by breathing. The patient already presents with mild
cardiomyopathy (ejection fraction of 55%, mild mitral and
tricuspid regurgitation), severe restrictive respiratory defect, requiring continuous BiPAP. The patient was markedly denourished (BMI of 12 kg/m2) and presented with nearly absent
breathing sounds on the right side. Chest radiography showed large
pneumothorax on the right side with no signs of tension. Drainage was performed. Despite initial success, recurrence of
pneumothorax occurred on the several attempts of clamping. A bronchopleural
fistula was suspected and operative treatment was considered. Considering the comorbidities, he was graded ASA IV with a difficult airway due to
macroglossia, limited neck and mandibular mobility. Oro-tracheal intubation was performed with slight sedation (
propofol, without neuromuscular blocks). Difficult airway anatomy (direct laryngoscopy - Cormack 4) successfully approached with a bougie and Mccoy blade. Fibreoptic intubation approach was immediately available in the operating room, if required. Total intravenous anaesthesia was decided (
remifentanil and
propofol, administered by continuous infusion, without
neuromuscular blockers). Volume controlled protective ventilation as used (tidal volume 6-8ml/kg, respiratory frequency of 14-16/ min; FiO2: 0,5). No bronchopleural
fistula was detected and
pleurodesis was performed with
biologic glue. Patient remained intubated and was transferred to the ICU for monitoring, having been discharged on the 2nd day to the ward. Despite this,
pneumothorax recurrence occurred, and surgery was performed again, using the same anaesthetic approach, this time with successful closure of the bronchopleural
fistula.
CONCLUSION: