[Propofol anesthesia for a patient with congenital myotonic dystrophy]

We report the anesthetic management for a five year old boy with congenital myotonic dystrophy. The patient was scheduled for bilateral orchiopexy under general anesthesia. Anesthesia was induced with fentanyl 50 micrograms, vecuronium 0.6 mg and propofol 40 mg intravenously to facilitate tracheal intubation. During operation, we monitored train of four ratio (TOF) to confirm effect of muscle relaxation. Anesthesia was maintained with propofol (2 mg.kg-1.hr-1), nitrous oxide and caudal block. At the end of the operation, the patient recovered smoothly from anesthesia and post-operative course was uneventful. Congenital myotonic dystrophy presents many problems for the management of general anesthesia, because of respiratory or circulatory complications. In this case, we were careful not to use drugs which may cause respiratory or circulatory depression. We have demonstrated that anesthesia with propofol is a safe method for the anesthetic management of a patient with this disease.