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Myotonic dystrophy, type 1 (Steinert's disease) is characterized by muscle weakness and wasting (most prominent in the cranial and distal limb musculature), periodic myotonia, progressive myopathy, insulin resistance, defects in cardiac conduction, neuropsychiatric impairment, cataracts, testicular atrophy, and frontal balding in males. Mitral valve prolapse may be found in 20% of patients. The majority of perioperative complications are pulmonary: smooth muscle atrophy, which leads to poor gastric motility, when coupled with a diminished cough reflex, promotes aspiration. Succinylcholine will produce contractions lasting for several minutes, thus making intubation and ventilation a challenge. Succinylcholine should be avoided. Patients are not resistant to nondepolarizing muscle relaxants and short-acting nondepolarizing muscle relaxants (or avoidance of relaxation) are therefore advised. If a nondepolarizing muscle relaxant is used, reversal of neuromuscular blockade is not contraindicated. Patients with myotonic dystrophy can be very sensitive to anesthetic agents, with hypersomnolence and CO2 retention sometimes being observed.