The diagnosis of mitochondrial
disease should be considered in any child with a multisystem neurological
disorder or who is being investigated for hypotonia. Anesthesia-related
morbidity and mortality risk is essentially linked to the preoperative
status of the child, that is, the number and extent of organ dysfunction.
Avoid any elective anesthesia/surgery in the presence of infection or
temperature because cytokines (mainly tumor necrosis factor) inhibit some
complex of the respiratory chain. The following should be carefully evaluated: CNS:
seizures, myoclonus, strokes, swallowing problems; metabolic: usual venous or arterial
concentration of lactates and glucose; muscles: hypotonia, contractures, scoliosis;
cardiac: even if the child is asymptomatic, ECG and echocardiography to exclude for
conduction disorders and cardiomyopathy, respectively; if a pacemaker has
been inserted, check it to be sure that it is functioning properly;
pulmonary: frequent infections, chronic aspiration, pulse oximetry breathing room air,
obstructive and/or central apnea; reduced ventilatory drive is common and
many patients have an abnormal response to both hypoxia and hypercarbia;
deaths have been reported following sedation with chloral hydrate or
diazepam; hepatic and renal function must be evaluated; nutritional status and diet (glucose and/or lipid rich),
tolerance to fasting and treatment (carnitine, vitamin Q, antiepileptic
drugs).