Carnitine Palmitoyl Transferase Type II (CPT II) deficiency is a disorder of fatty acid beta oxidation that causes decreased adenosine triphosphate (ATP) and ketone production during periods of fasting or high energy requirements. Labor and delivery can precipitate attacks for parturients with this disorder, causing hypoglycemia, muscle weakness, rhabdomyolysis, and kidney failure. Anesthetic management considers the delivery mode and anesthetic medications available to reduce these risks. We present the case of a pregnant patient with CPT II deficiency with labor epidural analgesia and a vaginal delivery, with alternative plans had a different delivery mode been required.
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