[Anesthesia for cesarean section in a case of Emery-Dreifuss muscular dystrophy]

We describe the anesthetic technique used in a patient with Emery-Dreifuss muscular dystrophy, a rare form whose genetic transmission is usually linked to the X-chromosome but which can be dominant, thus occasionally affecting women. A woman with vaginal condylomata was scheduled for elective cesarean. She presented proximal muscle weakness in the upper extremities, flexion contracture of both elbows and neck and, occasionally, respiratory difficulty. Surgery was performed with epidural anesthesia with no noteworthy complications. The anesthetic implications of this clinical picture depend on the likelihood of malignant hyperthermia, difficult intubation, arrhythmias or respiratory insufficiency due to muscle weakness. Continuous epidural or spinal anesthesia provides a more progressive level of blockade and greater control.