Anesthetic management in a patient with spinal progressive muscle atrophy and with compromised severe restrictive respiratory dysfunction

Spinal progressive muscular atrophy (SPMA) is a rare lower motor neuron disease in which anesthetic management is often difficult as a result of muscle weakness and hypersensitivity to neuromuscular blocking agents. A 43-year-old female patient with SPMA was scheduled to undergo laparotomy for ovarian teratoma. Her vital capacity was only 530 ml and % volume capacity was 22.2%, indicating a severe restrictive pulmonary disfunction. We selected general and epidural anesthesia for the surgery. Anesthesia was induced by propofol and the trachea was intubated with additional topical anesthesia. Spontaneous ventilation was kept during anesthesia without neuromuscular blocking agents, and systemic and epidural opioids were avoided to evade their respiratory depression. Because of severe spine scoliosis syndrome, we inserted an epidural catheter into the epidural space under radiographic monitoring for safety and precision. Postoperative pain control was stable using continuous epidural infusion of 0.2 % ropivacaine without neurological adverse effects. One day after surgery, the trachea was extubated and respiratory function did not aggravate. Despite the controversy surrounding the use of neuraxial blocks in motor neuron diseases, in this case, epidural anesthesia was useful to keep the respiratory function stable.