title: Anaesthetic management of a patient with Leigh's syndrome.
abstract: OBJECTIVE
  Leigh's syndrome, a progressive neurodegenerative disorder of infancy and childhood, is clinically characterized mainly by developmental delay, nervous system dysfunction and respiratory abnormalities such as aspiration, wheezing, breathing difficulties, gasping, hypoventilation and apnoea. Acute exacerbation and respiratory failure may follow surgery, general anaesthesia or intercurrent illnesses. Hyperlecithinemia is variably present. Histopathological findings include necrosis, vascular proliferation, astrocytosis and demyelination of several brain areas. We present a 30-month-old patient with Leigh's syndrome anaesthetized for extracorporeal shockwave lithotripsy, and describe the anaesthetic considerations.
  
  
  METHODS
  Leigh's syndrome was diagnosed at five months of age based on failure to thrive, lethargy, hypotonicity, choreo-athetosis and lactic acidaemia, with basal ganglia hypodense areas demonstrated by brain computerized tomographic scan. Muscle pyruvate dehydrogenase complex and NADH coenzyme Q oxidoreductase activity were 25% and 13% of control. No preoperative respiratory symptoms or signs were present. Preoperative fasting lasted two hours and gastric aspiration was negative. Anaesthesia was induced with ketamine and midazolam im, and N2O in oxygen, and maintained with propotol and N2O. No volatile anaesthetics were used. Intravenous fluids given were 1/2 normal saline and glucose 5% administered. Besides laryngospasm during anaesthetic induction, relieved by sublingual succinylcholine injection, the perianaesthetic course was uneventful. The lungs were mechanically ventilated and lithotripsy was performed. No adverse sequelae have occurred, and the patient was discharged one day later.
  
  
  CONCLUSIONS
  Perioperative management of patients with Leigh's syndrome requires cautious attention to the metabolic, neurological and respiratory aspects of the disease, and appropriate selection of anaesthetic drugs.
authors.affiliation: Department of Anesthesiology and CCM, Hadassah University Hospital, Jerusalem, Israel.
authors.forename: Z
authors.initials: Z
authors.lastname: Shenkman
authors.forename: I
authors.initials: I
authors.lastname: Krichevski
authors.forename: O N
authors.initials: ON
authors.lastname: Elpeleg
authors.forename: A
authors.initials: A
authors.lastname: Joseph
authors.forename: A
authors.initials: A
authors.lastname: Kadari
mesh_terms: D000768:Anesthesia, General; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007888:Leigh Disease / Q000150:complications* / Q000503:physiopathology; D008096:Lithotripsy; D016343:Monitoring, Intraoperative; D014514:Ureteral Calculi / Q000628:therapy