Leigh Syndrome is a rare mitochondial disorder. Affected patients require close monitoring during and after surgery.
(1) Tracheal irritability may be a hazard that may increase the risk for respiratory complications (aspiration, wheezing, gasping, hypoxia, etc).
(2) Cardiomyopathy with heart failure may complicate more severe forms.
(3) Spasticity may make positioning difficult and disturb the operative site.
(4) lactic acidosis
An alternative to general anesthesia should be considered when possible:
(1) deep sedation with propofol (limited to short term)
(2) spinal anesthesia
Infusion of lactated Ringer solution should be avoided.
Monitoring in the ICU should be considered and should include:
(1) body temperature
(2) heart rate
(3) blood pressure
(4) arterial PaO2 and oxygen saturation (to detect hypoxia and oxygen desaturation)
(5) end-tidal carbon dioxide
(6) blood glucose (to detect hypoglycemia)
(7) blood lactate
Kocamanoglu and Sarihasan recommended antibiotic prophylaxis to reduce the risk of postoperative pneumonia.
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Specialty: Anesthesiology, Pedatrics, Genetics