The diagnosis of mitochondrial
disease should be considered in any child with a multisystem neurologic
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. A
preoperative electrocardiogram should be considered at any age. The elevated
serum lactate resulting from defects in the respiratory chain necessitate
maintenance of a normal glucose level, normothermia, normocapnia, and more
stable possible cardiovascular function. Allelic variants are diabetic, so
serum glucose must be closely measured. Avoid any elective
anesthesia/ surgery in the presence of infection or raised
temperature because cytokines (mainly tumor-necrosis factor) inhibit some complex of the
respiratory chain. The following should be checked: central nervous system (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 conduction
disorders and cardiomyopathy, respectively; check functioning of pacemaker,
if present); 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, and deaths have been reported following sedation with
chloral hydrate or diazepam); hepatic and renal function; and nutritional status and diet (glucose and/or lipid-rich, tolerance to
fasting and treatment [carnitine, vitamin Q, antiepileptic drugs]).