Anaesthetic Management in a Duchennne Muscle Dystrophy Patient for Treatment of Recurrent Pneumothorax.

BACKGROUND

Duchenne muscular dystrophy (DMD) is an x linked recessive disorder. Long term prognosis is ominous, with development of respiratory distress and cardiomyopathy in advanced stage of the disease and expected death in the teens-to-mid 20s due to respiratory or cardiac failure. Peri-operative management of this patients is challenging due to difficult airway anatomy (macroglossia, limited neck and mandibular mobility). Additionally, they are at risk of developing malignant hyperthermia, rhabdomyolysis and hyperkalemic cardiac arrest when exposed to halogenated inhalational anaesthetics and depolarizing muscle relaxants.

METHODS

We describe a case of DMD proposed to a thoracotomy for treatment of recurrent pneumothorax and its anaesthetic approach.

RESULTS

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.

CONCLUSIONS

Total intravenous anaesthesia, without neuromuscular blockers, is a safe and effective option for DMD patients. Anaesthesiologists must consider the possibility of cardio-pulmonary disabilities, difficult airway management, as well as the high risk of malignant hyperthermia in these patients.