P009 Managing a trauma patient with myotonic dystrophy type-1

<h3></h3> <b>Please confirm that an ethics committee approval has been applied for or granted</b>: Not relevant (see information at the bottom of this page) <h3>Background and Aims</h3> 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. <h3>Results</h3> 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 <h3>Conclusions</h3> 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

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Published
2024-09-01
DOI
https://doi.org/10.1136/rapm-2024-esra.284
Primary Topic
Genetic Neurodegenerative Diseases
Type
article
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article

P009 Managing a trauma patient with myotonic dystrophy type-1

Bernardo Miguel, João Valente
Genetic Neurodegenerative Diseases
article

P009 Managing a trauma patient with myotonic dystrophy type-1

Bernardo Miguel, João Valente
article en

Abstract

<h3></h3> <b>Please confirm that an ethics committee approval has been applied for or granted</b>: Not relevant (see information at the bottom of this page) <h3>Background and Aims</h3> 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. <h3>Results</h3> 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 <h3>Conclusions</h3> 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

Instituto Superior Autónomo de Estudos Politécnicos (PT), Hospital de Santa Maria (PT)
Openalex Percentile: Top 83%
Genetic Neurodegenerative Diseases
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