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. Thirte en patients, median age 21 yr were anaesthetized by continuous propofol inf usion, fentanyl, atracurium and N2O to evaluate this combination in myotoni c dystrophy. Intraoperatively, neither exaggerated reactions nor haemodynam ic instability was observed. Recovery was smooth and quick. Although there was a significant decrease in mean postoperative vital capacity (965 (349) mi) from the preoperative value (1664 (566) ml, P=0.0028), there was no cha nge in mean postoperative Sp(O2) and there were no perioperative respirator y complications. Only two patients complained of nausea and vomiting. Simil arly, muscular hypertonia and shivering were not observed. We conclude that the combination of continuous propofol infusion and fentanyl was a success ful anaesthetic technique in these young myotonic dystrophy patients underg oing peripheral surgery.