Anesthetic management of the Prader Willi syndrome

Abstract

The principal features of PWS of concern to the anesthesiologist are the hypotonia and the disturbed carbohydrate fat metabolism. Extra attention should be given to airway protection and strength of cough, especially in the neonate. While not substantiated by the case described or reported by others yet, problems related to the use of muscle relaxants might be expected. The choice of anesthetic agent or technique should include consideration of the decreased muscle mass and greatly increased body fat content in these patients. Because these patients may continue to use circulating glucose to manufacture fat rather than to satisfy basal energy needs, the glucose level must be kept high enough during fasting states to allow vital organs to function properly even in the face of ongoing lipogenesis. Therefore, PWS patients should have glucose infusions during and after even brief surgical procedures; until oral intake is restarted, glucose levels should be monitored to prevent hypoglycemia and its possible complications.