Anaesthetic management of a patient with Leighs syndrome

~ r Leigh's syndrome, a progressive neurodegenerative disorder of infancy and childhood, is dinically characterized mainly by developmental delay, nervous system dysfunction and respiratory abnormalities such as aspiration, wheezing, breathing ditf~ulties, gasping, hypoventilation and apnoea. Acute exacerbation and respiratory failure may follow surgery, general anaesthesia or intercurrent illnesses. Hypedactataemia is variably present. Histopathologlcal findings indude necrosis, vascular proliferation, astrocytosis and demyelinafion of several brain areas. We present a 30-month-old patient ~ Leigh's syndrome anaest~zed for extracorporeal shockwave litf~tdpsy, and describe the anaesthetic considerations. tr'~linlcal features: Leigh's syrrlrome was diagnosed at f~e months of age based on failure to thrive, lethargy, hypotonicity, choreo-athetosis and lactic addaemia, with basal ganglia hypodense areas demonsbaled by brain computerized tomographic scan. Muscle p ~ dehydrogenase complex and NADHcoenzyme Q ~xidoreductase activity were 25% and 13% of control. No preoperative respiratory symptoms or signs were present. Preoperative fasting lasted two hours and gastric a s p i ~ was negat~e. Anaesthesia was induced with ketamine and midazolam ira, and N~O in oxygen, and maintained with propofol and N~O. No volatile anaesthetics were used. Intravenous fluids given were 89 normal saline and glucose 5% administered. Besides laryngospasm during anaesthetic indu~on, relieved by sublingual sucdnylcholine injection, the perianaesthe~ 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. Condmiom Perioperative management of patients with Lei~n's syndrome requires cautious attent~ to the rnetabolic, neurological and respiratory aspects of the disease, and appropriate selection of anaestf~c drugs.