ABSTRACT
Propionic acidemia (PA) is a rare, multi-systemic inborn error of metabolism. PA results from an impaired activity of the mitochondrial enzyme, propionyl-CoA carboxylase (PCC). PCC holds an essential role in the catabolic pathways for odd-chain fatty acids, cholesterol side-chains and branched-chain amino acids. Errors in these pathways result in the accumulation of toxic metabolites that may advance into end-organ damage and dysfunction. Clinical manifestations of PA include relapsing courses of severe metabolic acidosis, concurrent viral or bacterial infection, episodic vomiting, gastroesophageal reflux disease (GERD), seizures, developmental delay, hypotonia, hyperammonemia, osteopenia, pancreatitis and cardiomyopathy. This case describes a 3-year-old boy with PA who presented with an acute metabolic crisis, precipitated by Staphylococcus epidermidis (S. epidermidis) bacteremia and severe acute respiratory syndrome due to coronavirus 2 (SARS-CoV-2) co-infection. He required anesthetic management for surgical removal of an infected central venous port. Anesthetic care for this patient presented the unique challenges of metabolic decompensation amidst infection with SARS-CoV-2. Options for anesthetic care for patients with PA have been elucidated in the literature. However, to our knowledge, this is the first case to describe anesthetic management in a PA patient with SARS-CoV-2.