Procedural sedation for paediatric patients with spinal muscular atrophy undergoing intrathecal treatment.

Abstract

Nusinersen is administered intrathecally for treating spinal muscular atrophy (SMA). Procedural sedation is common with intrathecal treatment in children. The purpose of this study is to emphasize that intrathecal treatment of paediatric patients with SMA I, II and III can be tolerated with procedural sedation instead of general anaesthesia. Data were collected the from the anaesthesia charts and electronic medical records of 14 paediatric patients with SMA types I, II and III who underwent procedural sedation for repeated intrathecal treatments for SMA. Intravenous induction was performed, and patients were oxygenated with a face mask or nasal cannula while spontaneous breathing continued. Fourteen patients were included in the study: one SMA I, eight SMA II and five SMA III. They underwent 88 intrathecal nusinersen injections in total. In the one SMA I patient, of 8 months, the procedure was performed under local anaesthesia. In all other patients, the treatments were performed under procedural sedation. Different combinations of midazolam, ketamine, propofol, fentanyl and remifentanil were used. The mean doses of the agents used were 0.03 mg kg-1 , 0.97 mg kg-1 , 2.71 mg kg-1 , 0.84 μg kg-1 and 0.5μg kg-1 , respectively. There were no intraoperative or postoperative complications. We found the procedural sedation to be sufficient, safe and effective in SMA II and III paediatric patients who underwent nusinersen treatment intrathecally, provided anaesthetic agents are titrated and administered carefully.