Continuous propofol anaesthesia for patients with myotonic dystrophy.

Myotonic dystrophy, a rare genetic disorder, may pose a serious problem to the anaesthesiologist due to muscular and extramuscular involvement. Thirteen patients, median age 21 yr were anaesthetized by continuous propofol infusion, fentanyl, atracurium and N2O to evaluate this combination in myotonic dystrophy. Intraoperatively, neither exaggerated reactions nor haemodynamic instability was observed. Recovery was smooth and quick. Although there was a significant decrease in mean postoperative vital capacity (965 (349) ml) from the preoperative value (1664 (566) ml, P = 0.0028), there was no change in mean postoperative SpO2 and there were no perioperative respiratory complications. Only two patients complained of nausea and vomiting. Similarly, muscular hypertonia and shivering were not observed. We conclude that the combination of continuous propofol infusion and fentanyl was a successful anaesthetic technique in these young myotonic dystrophy patients undergoing peripheral surgery.