A patient with short-chain acyl-CoA dehydrogenase (SCAD) deficiency needs to be managed carefully during the perioperative period.
Hypoglycemia and hypovolemia are avoided by infusion of 5% dextrose in half normal saline with 20 mEq potassium chloride at maintenance rates
Myocardial dysfunction should be watched for. Intraoperative echocardiography can be used to monitor preload and cardiac function.
Things to avoid or to keep to a minimum:
(1) lactated Ringer's (liver may not be able to handle a lactate load)
(2) nondepolarizing neuromuscular blocking agents
(3) depolarizing agents
(4) hypovolemia
(5) propofol
Additional concerns:
(1) metabolic acidosis
(2) seizures
(3) cyclic vomiting
(4) hypotonia
(5) difficult intubation and airway maintenance
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Specialty: Anesthesiology, Pedatrics, Genetics
