ANESTHETIC MANAGEMENT IN MAPLE-SYRUP-URINE-DISEASE

Two children with maple syrup urine disease undergoing emergency and e lective surgery are reported. The administration of ketamine to a 12-m onth-old boy without neurological problems and propofol infusion to a 5-year-old girl with a history of convulsions, was uneventful. We beli eve that a full knowledge of the pathophysiology of maple syrup urine disease and potential peri-operative problems associated with the dise ase are essential, so that patients are not subjected to unnecessary r isks.