ESRA19-0382 Steinert’s disease – how the outcome is improved by regional anesthesia

Background and aims Steinert’s disease (SD) is the most common muscular dystrophy of the adult. It is a multi-systemic disorder characterized by skeletal muscle weakness and myotonia, respiratory, cardiac and others abnormalities. SD is a challenge for anesthesiologists due to the exquisite sensitivity to the anesthetics, resulting in potential postoperative complications (frequency of 8.2%) and the risk of a myotonic crisis in the perioperative period. This report aims to present a case of ulnar osteosynthesis in a patient with SD and discuss the possible role of regional anesthesia in the outcome of these patients. Methods A 49-year-old man, ASA III, with SD was scheduled for ulnar osteosynthesis. His past medical history was significant for hiatal hernia, obstructive sleep apnea and restrictive pulmonary disease. His only prior anesthetic exposure was laparoscopic cholecystectomy, with severe respiratory depression requiring intensive care unit admission postoperatively. An ultrasound-guided axillary brachial plexus block with 1.5% mepivacaine (150 mg) and 0.375% ropivacaine (37.5 mg) was performed. A forced-air warming blanket ensured normothermia. Results Intraoperatively the patient remained stable and comfortable. No intercurrences were recorded. Postoperative course was uneventful and the patient was discharged after 2 days. Conclusions Meticulous anaesthetic management in SD is crucial, as is the knowledge about its anesthetic implications and perioperative complications. With this in mind, regional anesthesia presented as a desirable alternative to general anesthesia, effective either in analgesia or in ventilatory support avoidance, in the postoperative period. In fact, the choice of the adequate anesthetic technique plays a decisive role in the outcome of the patient.