Episode 254: Keywords Part 22: Dystrophies and Trisomy 21
May 8, 2023
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Dr. Gillian Isaac discusses muscular dystrophies and trisomy 21, covering topics like anesthetic management strategies, risks for malignant hyperthermia, drug sensitivities, bradycardia in trisomy 21 patients, and Disney World trip mishaps.
Patients with muscular dystrophy are at risk of perioperative complications due to triggering agents like succinylcholine, requiring careful intraoperative management.
Understanding conditions associated with malignant hyperthermia susceptibility is crucial for safe anesthetic care in patients with specific disorders.
Deep dives
Patients with Muscular Dystrophy: Anesthetic Risks
Patients with muscular dystrophy, such as Duchenne and Becker muscular dystrophy, are at risk for perioperative complications due to triggering agents like succinylcholine that can induce skeletal muscle breakdown, leading to hyperkalemia and rhabdomyolysis. Intraperative management involves avoiding triggering agents and considering cardiac conduction abnormalities, hypersensitivity to anesthetics, and metabolic abnormalities.
Myotonic Dystrophy Anesthetic Considerations
Myotonic dystrophy, characterized by progressive muscle weakness and prolonged contractions, poses similar intraoperative risks as other muscular dystrophy types, including cardiac issues, aspiration, sensitivity to drugs, and metabolic abnormalities. Management may involve referring to specialized resources like the Myotonic Dystrophy Foundation's anesthetic management suggestions.
Malignant Hyperthermia Susceptibility
Patients with disorders like King-Denboros syndrome have an increased risk of malignant hyperthermia compared to the general population. Understanding conditions associated with malignant hyperthermia susceptibility is crucial for anesthetists to provide safe care during procedures.
Cardiac Evaluation in Duchenne Muscular Dystrophy
In patients with Duchenne muscular dystrophy presenting for procedures like gastric tube placement, evaluating for dilated cardiomyopathy and valve regurgitation is essential due to potential cardiac complications even in the absence of significant skeletal muscle weakness. Understanding the cardiac implications in these patients is crucial for preoperative assessment and management.