Epidural anaesthesia in a child with possible spinal muscular atrophy

Abstract

Spinal muscular atrophy (SMA) is a rare lower motor neurone disease in which anaesthetic management is often difficult as a result of muscle weakness and hypersensitivity to neuromuscular blocking agents. Neuraxial anaesthesia is controversial in these patients; however, some cases have been reported in which neuraxial anaesthesia has been used without neurological sequelae. We describe a 7-year-old patient with possible SMA scheduled for a Grice-arthrodesis. Because of previous prolonged postoperative drowsiness and poor oral intake, we decided to use an epidural technique with sevoflurane sedation and spontaneous ventilation to avoid the use of muscle relaxants and systemic opioids and consequently admission to the intensive care unit. After 3 days, the epidural analgesia was stopped and the patient regained her preoperative motor function within 5 h. Despite the controversy surrounding the use of neuraxial techniques in neuromuscular disease, we found no well-founded basis for this in patients with SMA in the literature.