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Background and Aims Myotonic dystrophy type-1 is a multisystemic disease with autosomal dominant inheritance which is associated with muscle weakness, periodic myotonia and cardiac conduction abnormalities. These patients are at increased risk of perioperative respiratory complications due to aspiration of gastric content and post-operative worsening of underlying restrictive lung disease. Although there is no association with malignant hyperthermia, anaesthesiologists should avoid myotonic crisis triggers such as hypothermia, shivering, pain and succinylcholine. We aim to discuss anaesthetic options for a unique case of a myotonic dystrophy type-1 patient proposed for external fixation of lower limb bilateral trauma fractures.

Results 15-year-old male, with a background of myotonic dystrophy type-1, victim of a high impact pedestrian-car collision resulting in multiple injuries (right lung contusion, bilateral femoral shaft fractures and bilateral pubic rami fractures), to be submitted to external fixation of lower limb fractures. The patient was carefully positioned with the cooperation of orthopaedists in a left lateral decubitus position, and a combined spinal and epidural anaesthetic technique was performed. The surgery was uneventful and postoperative pain management was guaranteed with continuous epidural infusion of ropivacaine

Conclusions The complexity of this case is related to the risks associated with myotonic dystrophy type-1 combined with the injuries caused by trauma. To minimize postoperative respiratory complications, the priority was to minimize opioids, induction agents and avoid neuromuscular blockers to maintain spontaneous ventilation. The bilaterality of fractures made mobilization more complex, but ultimately, and after discussion with the orthopaedic team, a left lateral decubitus was deemed safe to perform a neuraxial technique