Anesthetic Management of a Patient with Isovaleric Acidemia

Abstract

A 3-year-old male with isovaleric acidemia presented for dental rehabilitation under general anesthesia. In times of stress, such as in the perioperative period, patients with isovaleric acidemia are at greater risk for morbidity and mortality from disordered metabolism, including glucose disturbances, hyperammonemia, hypocalcemia, and non-anion gap metabolic acidosis. Communication between the anesthesiology, dental, and endocrine teams allowed for safe and successful care of the patient.