[Anaesthesia for combined pancreatic and renal transplantation in a patient with mitochondrial encepahalomyopathy--a case report].

Mitochondrial disorders encompass a group of syndromes produced by genetic defects that disrupt mitochondrial energy production. The impaired mitochondrial energy supply affects nearly all organs and tissues leading to a variable clinical presentation. The possible multisystem involvement complicates the management of anaesthesia and perioperative care. Exact knowledge of the path physiology of mitochondrial diseases may help to avoid perioperative anaesthesiological complications. This report describes the anaesthetic management of a patient with a mitochondrial disorder during combined pancreatic and renal transplantation, and discusses some of the anaesthetic implications of mitochondrial diseases. Due to the potential susceptibility of patients with mitochondrial diseases to malignant hyperthermia, anaesthesia was induced and maintained as total intravenous anaesthesia using propofol, alfentanil and cis-atracurium. In addition, the patient was treated intraoperatively with hydrocortisone (initial bolus of 50 mg followed by a continuous infusion of 4.8 mg/h) and insulin (continuous infusion of 2 IE/h) in order to manage the adrenocortical insufficiency as well as to treat the diabetes mellitus. Using this anaesthetic technique, satisfactory haemodynamic and metabolic conditions were achieved during surgery. The postoperative period, however, was marked by severe respiratory complications.