THE ANESTHETIC MANAGEMENT OF A PATIENT WITH EMERY-DREIFUSS MUSCULAR-DYSTROPHY

Purpose: This case report presents a patient with Emery-Dreifuss Muscu lar Dystrophy and describes the anaesthetic considerations. Clinical f eatures: The features of Emery-Dreifuss Muscular Dystrophy are contrac tures, humeroperoneal muscle weakness and cardiomyopathy. The anaesthe tic considerations for this syndrome are difficult tracheal intubation , difficult spinal anaesthetic, heart block, gastric reflux, rhabdomyo lysis, and unproved malignant hyperthermia susceptibility. Conclusion: The major anaesthetic problem for the patient with Emery-Dreifuss Mus cular Dystrophy could be a life-threatening cardiomyopathy.