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Clinical Anesthesiology II pp 167–191Cite as

Anesthetic Implications of Duchenne Muscular Dystrophy and the Surgical Repair of Scoliosis

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Abstract

Duchenne muscular dystrophy (DMD) is the most common hereditary neuromuscular disease worldwide. While there is no cure for this progressive and debilitating disease, improvements in the supportive care of patients with DMD have significantly improved their life expectancy, thus making it a disease that can be encountered outside of pediatric clinical settings. DMD is caused by an X-linked recessive mutation in the gene coding for dystrophin, an integral protein in the cell membrane and cytoskeleton of muscle cells. Besides significant muscle weakness, medical conditions associated with DMD include cardiomyopathy, respiratory failure, restrictive lung disease, and scoliosis. Anesthetic management of patients with DMD includes a thorough preoperative evaluation, the availability of advanced airway equipment, aspiration precautions, and careful attention to positioning. While patients with DMD are not at risk for malignant hyperthermia specifically, they are at risk for anesthesia-induced reactions including hyperkalemia, rhabdomyolysis, and cardiac arrest without other signs of a hypermetabolic state. Therefore, malignant hyperthermia precautions are recommended. Spinal fusion is a common major surgery that patients with DMD require. The risk of surgical complications such as neurological injury and blood loss is higher for patients with neuromuscular scoliosis than those with idiopathic scoliosis due to the need for longer and more complex surgeries to fuse more levels of the spine. Motor and sensory neuromonitoring is the standard of care for these procedures even though the quality of baseline signals may be difficult to obtain due to muscle weakness. Techniques to minimize blood loss and allogenic transfusions in high-risk patients include administration of epoetin alfa, autologous donation, acute normovolemic hemodilution, controlled hypotension, intrathecal morphine administration, cell saver, and antifibrinolytic agents. Anesthesiologists may also consider indices to guide volume resuscitation in scoliosis surgery such as variation in aortic blood flow on echocardiography and esophageal Doppler monitoring.

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Authors and Affiliations

  1. Department of Anesthesiology, Southern California Permanente Medical Group, San Diego, CA, USA

    Sonya M. Seshadri

  2. Department of Anesthesiology, University of California, San Diego, CA, USA

    Karim T. Rafaat & Alyssa Brzenski

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  1. Sonya M. Seshadri
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Correspondence to Karim T. Rafaat .

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  1. Department of Anesthesiology, University of California, UCSD Medical Center, UCSD School of Medicine, San Diego, CA, USA

    Jonathan L. Benumof

  2. Department of Anesthesiology, University of California, UCSD Medical Center, UCSD School of Medicine, San Diego, CA, USA

    Gerard R. Manecke

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Seshadri, S.M., Rafaat, K.T., Brzenski, A. (2019). Anesthetic Implications of Duchenne Muscular Dystrophy and the Surgical Repair of Scoliosis. In: Benumof, J., Manecke, G. (eds) Clinical Anesthesiology II. Springer, Cham. https://doi.org/10.1007/978-3-030-12365-9_9

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