General anesthesia combined with rectus sheath block for an open jejunostomy in a patient with amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis（ALS）is a rare neurodegenerative disease that affects the upper and lower motor neurons and eventually causes, systemic muscle atrophy, paralysis, and bulbar paralysis. Furthermore, weakness of the respiratory muscles leads to respiratory failure and death. The perioperative management of patients with ALS requires the prevention of respiratory complications and avoidance of decline in respiratory function. We performed general anesthesia with rectus sheath block（RSB）to create an open jejunostomy in patients with ALS. Thereby, we managed the perioperative period without any respiratory complications. In this report, we discuss the benefits and problems of general anesthesia with regional anesthesia for patients with ALS complications, along with discussing the existing literature. The current patient had dysphagia, difficulty in excreting sputum, and severe dyspnea, and thus was considered to be at a high risk of developing respiratory complications. Choosing muscle relaxants, opioids, spinal trunk anesthesia, or peripheral nerve block is challenging. Particularly, the use of nondepolarizing muscle relaxants in ALS is often discouraged due to increased sensitivity to muscle relaxants, uncertainty of muscle relaxation monitoring, and reports of recurarization after sugammadex reversal. Additionally, postoperative analgesia should be considered, due to concerns regarding opioid-induced respiratory depression and delayed recovery of spontaneous respiration. We performed a single RSB preoperatively to achieve intraoperative muscle relaxation. Thereafter, we inserted a catheter for continuous RSB for postoperative analgesia. We noted that intraoperative use of muscle relaxants and opioids could be reduced, and postoperative analgesia was achieved at rest. However, the effects of either of these alone were insufficient. There are limitations regarding the types and doses of medications that can be safely used in ALS, since the progression of the disease and the degree of decline in respiratory function can vary in ALS. Careful perioperative management and collaboration with surgeons is required, considering each patientʼs condition and surgical invasiveness. Using RSB for multimodal anesthesia and postoperative management may be an effective tool in this regard.