Resistance to rocuronium in a patient with propionic acidemia undergoing appendectomy for acute appendicitis: A case report with literature review

Abstract

Propionic acidemia is a rare metabolic disorder caused by a deficiency of the mitochondrial enzyme, propionyl-CoA carboxylase, which is required in the catabolic pathways of several amino acids, odd-chain fatty acids and the side chains of cholesterol. Clinically, the disorder may present with multisystemic manifestations including metabolic ketoacidosis, hyperlactatemia, hyperammonemia, developmental delay and neurological dysfunction. The key principle in managing or preventing acute exacerbations consists of protein restricted diet and avoidance of factors that may potentiate relevant catabolic pathways. We describe an unusual case of a female patient with propionic acidemia undergoing appendectomy for acute appendicitis, who was resistant to a nondepolarizing neuromuscular blocker (rocuronium). In literature, there has been reported cases of resistance to nondepolarizing neuromuscular blockers associated with certain pathologic conditions or medications, but such resistance has not been previously described in the context of propionic acidemia. Rocuronium resistance in our index patient could be attributable to a sum of potential etiologic factors including the use of valproic acid, and upper motor neuron damage, a rare complication of chronic propionic acidemia.